Clinical Interests

Immunobiology; translational research; immunologic methods development

Research Interests

Clinical investigation of primary immunodeficiency disorders (with emphasis on disorders of immunodysregulation and B-cell disorders); immuno-reconstitution following blood and marrow transplantation; diagnostic immunology (with emphasis on flow cytometry) 

Jodele S, Bleesing JJ, Mehta PA, Filipovich AH, Laskin BL, Goebel J, Pinkard SL, Davies SM. Successful early intervention for hyperacute transplant-associated thrombotic microangiopathy following pediatric hematopoietic stem cell transplantation. Pediatr Transplant. 2010 Nov 5.

Eckrich MJ, Yang E, Domm J, Ho R, Calder C, Manes B, Bleesing J, Frangoul H. A Unique Clinical Presentation of X-Linked Lymphoproliferative Syndrome With a Novel Mutation in SH2D1A and Review of the Literature. J Pediatr Hematol Oncol. 2010 Oct 21. 

Marsh RA, Bleesing JJ, Filipovich AH. Using flow cytometry to screen patients for X-linked lymphoproliferative disease due to SAP deficiency and XIAP deficiency. J Immunol Methods. 2010 Oct 31;362(1-2):1-9. 

Marsh RA, Villanueva J, Kim MO, Zhang K, Marmer D, Risma KA, Jordan MB, Bleesing JJ, Filipovich AH. Patients with X-linked lymphoproliferative disease due to BIRC4 mutation have normal invariant natural killer T-cell populations. Clin Immunol. 2009 Jul;132(1):116-23. 

Marsh RA, Villanueva J, Zhang K, Snow AL, Su HC, Madden L, Mody R, Kitchen B, Marmer D, Jordan MB, Risma KA, Filipovich AH, Bleesing JJ. A rapid flow cytometric screening test for X-linked lymphoproliferative disease due to XIAP deficiency. Cytometry B Clin Cytom. 2009 Sep;76(5):334-44.

Hansen MD, Filipovich AH, Davies SM, Mehta P, Bleesing J, Jodele S, Hayashi R, Barnes Y, Shenoy S. Allogeneic hematopoietic cell transplantation (HCT) in Hurler's syndrome using a reduced intensity preparative regimen. Bone Marrow Transplant. 2008 Feb;41(4):349-53.

Bhatla D, Davies SM, Shenoy S, Harris RE, Crockett M, Shoultz L, Smolarek T, Bleesing J, Hansen M, Jodele S, Jordan M, Filipovich AH, Mehta PA. Reduced-intensity conditioning is effective and safe for transplantation of patients with Shwachman-Diamond syndrome. Bone Marrow Transplant. 2008 Aug;42(3):159-65.

Zhang K, Johnson JA, Biroschak J, Villanueva J, Lee SM, Bleesing JJ, Risma KA, Wenstrup RJ, Filipovich AH. Familial haemophagocytic lymphohistiocytosis in patients who are heterozygous for the A91V perforin variation is often associated with other genetic defects. Int J Immunogenet. 2007 Aug;34(4):231-3. 

Book Chapters

JJH Bleesing. Assays for B Cell and Germinal Center Development (unit 7.34). In Current Protocols in Immunology. JE Coligan, BE Bierer, DH Margulies, EM Shevach, W Strober (eds.). Hoboken: John Wiley & Sons, 2004.

JJH Bleesing, TA Fleisher, JM Puck. Autoimmune lymphoproliferative syndrome (ALPS). In Immunologic Disorders in Infants and Children. 5th ed. ER Stiehm, HD Ochs, JA Winkelstein (eds). Philadelphia, PA: WB Saunders, 2004.

MBBS: University of Newcastle-Upon-Tyne, England, 1981.

Clinical and Fellowship Training: The Royal Victoria Infirmary, Newcastle General Hospital and Great Ormond St. Hospital, 1981 to 1985.

PhD: University of Newcastle-Upon-Tyne, England, 1989.

Pediatric Fellowship: University of Minnesota, Minneapolis, MN, 1989 to 1993.

Mehta PA, Harris RE, Davies SM, Kim MO, Mueller R, Lampkin B, Mo J, Myers K, Smolarek TA. Numerical chromosomal changes and risk of development of myelodysplastic syndrome--acute myeloid leukemia in patients with Fanconi anemia. Cancer Genet Cytogenet. 2010 Dec;203(2):180-6.

Jodele S, Bleesing JJ, Mehta PA, Filipovich AH, Laskin BL, Goebel J, Pinkard SL, Davies SM.Successful early intervention for hyperacute transplant-associated thrombotic microangiopathy following pediatric hematopoietic stem cell transplantation. Pediatr Transplant. 2010 Nov 5.

Marsh RA, Vaughn G, Kim MO, Li D, Jodele S, Joshi S, Mehta PA, Davies SM, Jordan MB, Bleesing JJ, Filipovich AH. Reduced-intensity conditioning significantly improves survival of patients with hemophagocytic lymphohistiocytosis undergoing allogeneic hematopoietic cell transplantation. Blood. 2010 Dec 23;116(26):5824-31.

Laskin BL, Goebel J, Davies SM, Khoury JC, Bleesing JJ, Mehta PA, Filipovich AH, Paff ZN, Lawrence JM, Yin HJ, Pinkard SL, Jodele S. Early clinical indicators of transplant-associated thrombotic microangiopathy in pediatric neuroblastoma patients undergoing auto-SCT. Bone Marrow Transplant. 2010 Aug 9.

Phillips CL, Gerbing R, Alonzo T, Perentesis JP, Harley IT, Meshinchi S, Bhatla D, Radloff G, Davies SM. MDM2 polymorphism increases susceptibility to childhood acute myeloid leukemia: a report from the Children's Oncology Group. Pediatr Blood Cancer. 2010 Aug;55(2):248-53.

Mehta PA, Vinks AA, Filipovich A, Bleesing J, Jodele S, Jordan MB, Marsh R, Tarin R, Edwards S, Fearing D, Lawrence J, Davies SM. Alternate-day micafungin antifungal prophylaxis in pediatric patients undergoing hematopoietic stem cell transplantation: a pharmacokinetic study. Biol Blood Marrow Transplant. 2010 Oct;16(10):1458-62.

Sakagami T, Beck D, Uchida K, Suzuki T, Carey BC, Nakata K, Keller G, Wood RE, Wert SE, Ikegami M, Whitsett JA, Luisetti M, Davies S, Krischer JP, Brody A, Ryckman F, Trapnell BC.Patient-derived granulocyte/macrophage colony-stimulating factor autoantibodies reproduce pulmonary alveolar proteinosis in nonhuman primates. Am J Respir Crit Care Med. 2010 Jul 1;182(1):49-61.

Wang D, Zhang W, Kalfa TA, Grabowski G, Davies S, Malik P, Pan D. Reprogramming erythroid cells for lysosomal enzyme production leads to visceral and CNS cross-correction in mice with Hurler syndrome. Proc Natl Acad Sci U S A. 2009 Nov 24;106(47):19958-63.

Davies SM, Wang D, Wang T, Arora M, Ringden O, Anasetti C, Pavletic S, Casper J, Macmillan ML, Sanders J, Wall D, Kernan NA. Recent decrease in acute graft-versus-host disease in children with leukemia receiving unrelated donor bone marrow transplants. Biol Blood Marrow Transplant. 2009 Mar;15(3):360-6.

Myers KC, Davies SM. Hematopoietic stem cell transplantation for bone marrow failure syndromes in children. Biol Blood Marrow Transplant. 2009 Mar;15(3):279-92. Review.

Clinical Interests

Primary immunodeficiencies; BMT for primary immunodeficiencies; hemophagocytic lymphocytosis; post-BMT immune reconstruction 

Research Interests

Biology and genetics of hemophagocytic lymphohistiocytosis; investigation of hematopoietic cell transplantation for genetic immunodeficiencies; immunoreconstitution following pediatric stem cell transplantation 

Jodele S, Bleesing JJ, Mehta PA, Filipovich AH, Laskin BL, Goebel J, Pinkard SL, Davies SM. Successful early intervention for hyperacute transplant-associated thrombotic microangiopathy following pediatric hematopoietic stem cell transplantation.  Pediatr Transplant. 2010 Nov 5.

Marsh RA, Vaughn G, Kim MO, Li D, Jodele S, Joshi S, Mehta PA, Davies SM, Jordan MB, Bleesing JJ, Filipovich AH.  Reduced-intensity conditioning significantly improves survival of patients with hemophagocytic lymphohistiocytosis undergoing allogeneic hematopoietic cell transplantation. Blood. 2010 Dec 23;116(26):5824-31.

Marsh RA, Bleesing JJ, Filipovich AH. Using flow cytometry to screen patients for X-linked lymphoproliferative disease due to SAP deficiency and XIAP deficiency.J Immunol Methods. 2010 Oct 31;362(1-2):1-9.

Laskin BL, Goebel J, Davies SM, Khoury JC, Bleesing JJ, Mehta PA, Filipovich AH, Paff ZN, Lawrence JM, Yin HJ, Pinkard SL, Jodele S. Early clinical indicators of transplant-associated thrombotic microangiopathy in pediatric neuroblastoma patients undergoing auto-SCT.  Bone Marrow Transplant. 2010 Aug 9.

Filipovich AH, Zhang K, Snow AL, Marsh RA. X-linked lymphoproliferative syndromes: brothers or distant cousins? Blood. 2010 Nov 4;116(18):3398-408.

Marsh RA, Madden L, Kitchen BJ, Mody R, McClimon B, Jordan MB, Bleesing JJ, Zhang K, Filipovich AH. XIAP deficiency: a unique primary immunodeficiency best classified as X-linked familial hemophagocytic lymphohistiocytosis and not as X-linked lymphoproliferative disease. Blood. 2010 Aug 19;116(7):1079-82.

Marsh RA, Satake N, Biroschak J, Jacobs T, Johnson J, Jordan MB, Bleesing JJ, Filipovich AH, Zhang K. STX11 mutations and clinical phenotypes of familial hemophagocytic lymphohistiocytosis in North America. Pediatr Blood Cancer. 2010 Jul 15;55(1):134-40.

Filipovich AH. Hemophagocytic lymphohistiocytosis (HLH) and related disorders. Hematology Am Soc Hematol Educ Program. 2009:127-31.

Nicolaou SA, Neumeier L, Takimoto K, Lee SM, Duncan HJ, Kant SK, Mongey AB, Filipovich AH, Conforti L. Differential calcium signaling and Kv1.3 trafficking to the immunological synapse in systemic lupus erythematosus. Cell Calcium. 2010 Jan;47(1):19-28.

Marsh RA, Villanueva J, Kim MO, Zhang K, Marmer D, Risma KA, Jordan MB, Bleesing JJ, Filipovich AH. Patients with X-linked lymphoproliferative disease due to BIRC4 mutation have normal invariant natural killer T-cell populations. Clin Immunol. 2009 Jul;132(1):116-23.

Jubert C, Wall DA, Grimley M, Champagne MA, Duval M. Engraftment of unrelated cord blood after reduced-intensity conditioning regimen in children with refractory neuroblastoma: a feasibility trial. Bone Marrow Transplant. 2011 Feb;46(2):232-7.

 Jacobson PA, Huang J, Wu J, Kim M, Logan B, Alousi A, Grimley M, Bolaños-Meade J, Ho V, Levine JE, Weisdorf D. Mycophenolate pharmacokinetics and association with response to acute graft-versus-host disease treatment from the Blood and Marrow Transplant Clinical Trials Network. Biol Blood Marrow Transplant. 2010 Mar;16(3):421-9.

Jacobsohn DA, Gilman AL, Rademaker A, Browning B, Grimley M, Lehmann L, Nemecek ER, Thormann K, Schultz KR, Vogelsang GB. Evaluation of pentostatin in corticosteroid-refractory chronic graft-versus-host disease in children: a Pediatric Blood and Marrow Transplant Consortium study. Blood. 2009 Nov 12;114(20):4354-60.

Pulsipher MA, Wall DA, Grimley M, Goyal RK, Boucher KM, Hankins P, Grupp SA, Bunin N. A phase I/II study of the safety and efficacy of the addition of sirolimus to tacrolimus/methotrexate graft versus host disease prophylaxis after allogeneic haematopoietic cell transplantation in paediatric acute lymphoblastic leukaemia (ALL). Br J Haematol. 2009 Dec;147(5):691-9.

Pulsipher MA, Boucher KM, Wall D, Frangoul H, Duval M, Goyal RK, Shaw PJ, Haight AE, Grimley M, Grupp SA, Kletzel M, Kadota R. Reduced-intensity allogeneic transplantation in pediatric patients ineligible for myeloablative therapy: results of the Pediatric Blood and Marrow Transplant Consortium Study ONC0313. Blood. 2009 Aug 13;114(7):1429-36.

Chan KW, McDonald L, Lim D, Grimley MS, Grayson G, Wall DA. Unrelated cord blood transplantation in children with idiopathic severe aplastic anemia. Bone Marrow Transplant. 2008 Nov;42(9):589-95.  

Chan KW, Grimley MS, Taylor C, Wall DA. Early identification and management of graft failure after unrelated cord blood transplantation. Bone Marrow Transplant. 2008 Jul;42(1):35-41.

Swinney RM, Wall DA, Thomas PJ, Grimley MS, Taylor C, Tomlinson GE. Familial childhood leukemia cluster with multiple aggressive early-onset hematological malignancies. Leuk Lymphoma. 2006 May;47(5):930-2.

Shaughnessy PJ, Bachier C, Grimley M, Freytes CO, Callander NS, Essell JH, Flomenberg N, Selby G, Lemaistre CF. Denileukin diftitox for the treatment of steroid-resistant acute graft-versus-host disease. Biol Blood Marrow Transplant. 2005 Mar;11(3):188-93.

Parker A, Anderson C, Weiss KL, Grimley M, Sorrells D. Eukaryotic initiation factor 4E staining as a clinical marker in pediatric neuroblastoma. J Pediatr Hematol Oncol. 2004 Aug;26(8):484-7.

Clinical Interests

Bone marrow failure syndromes such as Fanconi anemia, Shwachman Diamond Syndrome, Dyskeratosis Congenita, Congenital Amegakaryocytic Thrombocytopenia, Diamond Blackfan Anemia, and severe aplastic anemia

Research Interests

Transplantation for children with bone marrow failure syndromes (Fanconi anemia, Shwachman Diamond syndrome, dyskeratosis congenita, amegakaryocytic thrombocytopenia, Diamond Blackfan anemia) and aplastic anemia. Cytogenetic abnormalities in Fanconi anemia.

Mehta PA, Harris RE, Davies SM, Kim MO, Mueller R, Lampkin B, Mo J, Myers K, Smolarek TA. Numerical chromosomal changes and risk of development of myelodysplastic syndrome--acute myeloid leukemia in patients with Fanconi anemia. Cancer Genet Cytogenet. 2010 Dec;203(2):180-6.

Harris RE, Termuhlen AM, Smith LM, Lynch J, Henry MM, Perkins SL, Gross TG, Warkentin P, Vlachos A, Harrison L, Cairo MS. Autologous Peripheral Blood Stem Cell Transplantation in Children with Refractory or Relapsed Lymphoma: Results of Children's Oncology Group Study A5962. Biol Blood Marrow Transplant. 2010 Jul 14.

Bhatla D, Davies SM, Shenoy S, Harris RE, Crockett M, Shoultz L, Smolarek T, Bleesing J, Hansen M, Jodele S, Jordan M, Filipovich AH, Mehta PA. Reduced-intensity conditioning is effective and safe for transplantation of patients with Shwachman-Diamond syndrome. Bone Marrow Transplant. 2008 Aug;42(3):159-65.

Farzin A, Davies SM, Smith FO, Filipovich A, Hansen M, Auerbach AD, Harris RE. Matched sibling donor haematopoietic stem cell transplantation in Fanconi anaemia: an update of the Cincinnati Children's experience. Br J Haematol. 2007 Feb;136(4):633-40.

Kelly PF, Radtke S, von Kalle C, Balcik B, Bohn K, Mueller R, Schuesler T, Haren M, Reeves L, Cancelas JA, Leemhuis T, Harris R, Auerbach AD, Smith FO, Davies SM, Williams DA. Stem cell collection and gene transfer in Fanconi anemia. Mol Ther. 2007 Jan;15(1):211-9.

Mehta PA, Ileri T, Harris RE, Williams DA, Mo J, Smolarek T, Auerbach AD, Kelly P, Davies SM. Chemotherapy for myeloid malignancy in children with Fanconi anemia. Pediatr Blood Cancer. 2007 Jun 15;48(7):668-72.

Boyer MW, Gross TG, Loechelt B, Leemhuis T, Filipovich A, Harris RE. Low risk of graft-versus-host disease with transplantation of CD34 selected peripheral blood progenitor cells from alternative donors for Fanconi anemia. J Pediatr Hematol Oncol. 2003 Nov;25(11):890-5.

Parshionikar SU, Willian-True S, Fout GS, Robbins DE, Seys SA, Cassady JD, Harris R. Waterborne outbreak of gastroenteritis associated with a norovirus. Appl Environ Microbiol. 2003 Sep;69(9):5263-8.

Harris RE. Reduction of toxicity of marrow transplantation in children with Fanconi anemia. J Pediatr Hematol Oncol. 1999 May-J un;21(3):175-6. No abstract available.

Harris RE, Sather HN, Feig SA. High-dose cytosine arabinoside and L-asparaginase in refractory acute lymphoblastic leukemia: the Children's Cancer Group experience. Med Pediatr Oncol. 1998 Apr;30(4):233-9.

MD: Vilnius University School of Medicine, Vilnius University School of Medicine, 1988-1994.

Residency: Pediatrics, Downstate HSC at Brooklyn, Brooklyn, NY, 1998-2001.

Fellowship: Pediatric Hematology Oncology, Childrens Hospital Los Angeles and Saban Research Institute, Keck School of Medicine University of Southern California, Los Angeles, CA, 2001-2004.

Certifications: Pediatrics, 2001; Pediatric Hematology / Oncology, 2004.

Licenses: California, 2001-present; Ohio, 2004-present.

Sanchez-Pinto LN, Laskin BL, Jodele S, Hummel TR, Yin HJ, Goebel J. BK virus nephropathy in a pediatric autologous stem-cell transplant recipient. Pediatr Blood Cancer. 2011 Mar;56(3):495-7.

Smith AR, Majhail NS, Macmillan ML, Defor TE, Jodele S, Lehmann LE, Krance R, Davies SM. Hematopoietic cell transplantation comorbidity index predicts transplant outcomes in pediatric patients. Blood. 2011 Jan 12

Marsh RA, Vaughn G, Kim MO, Li D, Jodele S, Joshi S, Mehta PA, Davies SM, Jordan MB, Bleesing JJ, Filipovich AH. Reduced-intensity conditioning significantly improves survival of patients with hemophagocytic lymphohistiocytosis undergoing allogeneic hematopoietic cell transplantation. Blood. 2010 Dec 23;116(26):5824-31.

Jodele S, Bleesing JJ, Mehta PA, Filipovich AH, Laskin BL, Goebel J, Pinkard SL, Davies SM. Successful early intervention for hyperacute transplant-associated thrombotic microangiopathy following pediatric hematopoietic stem cell transplantation. Pediatr Transplant. 2010 Nov 5.

Mehta PA, Vinks AA, Filipovich A, Bleesing J, Jodele S, Jordan MB, Marsh R, Tarin R, Edwards S, Fearing D, Lawrence J, Davies SM. Alternate-day micafungin antifungal prophylaxis in pediatric patients undergoing hematopoietic stem cell transplantation: a pharmacokinetic study. Biol Blood Marrow Transplant. 2010 Oct;16(10):1458-62.

Laskin BL, Goebel J, Davies SM, Khoury JC, Bleesing JJ, Mehta PA, Filipovich AH, Paff ZN, Lawrence JM, Yin HJ, Pinkard SL, Jodele S. Early clinical indicators of transplant-associated thrombotic microangiopathy in pediatric neuroblastoma patients undergoing auto-SCT. Bone Marrow Transplant. 2010 Aug 9.

Rayburg M, Davies SM, Mehta PA, Crockett M, Jodele S. Successful reduced-intensity bone marrow transplantation in a patient with bone marrow failure associated with Seckel syndrome. Br J Haematol. 2008 Aug;142(4):675-6.

Bhatla D, Davies SM, Shenoy S, Harris RE, Crockett M, Shoultz L, Smolarek T, Bleesing J, Hansen M, Jodele S, Jordan M, Filipovich AH, Mehta PA. Reduced-intensity conditioning is effective and safe for transplantation of patients with Shwachman-Diamond syndrome. Bone Marrow Transplant. 2008 Aug;42(3):159-65.

Hansen MD, Filipovich AH, Davies SM, Mehta P, Bleesing J, Jodele S, Hayashi R, Barnes Y, Shenoy S. Allogeneic hematopoietic cell transplantation (HCT) in Hurler's syndrome using a reduced intensity preparative regimen. Bone Marrow Transplant. 2008 Feb;41(4):349-53.

Jodele S, Blavier L, Yoon JM, DeClerck YA. Modifying the soil to affect the seed: role of stromal-derived matrix metalloproteinases in cancer progression. Cancer Metastasis Rev. 2006 Mar;25(1):35-43.

Clinical Interests

Histiocytic disorders: HLH and LCH

Research Interests

Better understanding histiocytic disorders and developing novel therapies for them; regulation of the immune response; immunotherapy of cancer

Marsh RA, Vaughn G, Kim MO, Li D, Jodele S, Joshi S, Mehta PA, Davies SM, Jordan MB, Bleesing JJ, Filipovich AH. Reduced-intensity conditioning significantly improves survival of patients with hemophagocytic lymphohistiocytosis undergoing allogeneic hematopoietic cell transplantation. Blood. 2010 Dec 23;116(26):5824-31.

Mehta PA, Vinks AA, Filipovich A, Bleesing J, Jodele S, Jordan MB, Marsh R, Tarin R, Edwards S, Fearing D, Lawrence J, Davies SM. Alternate-day micafungin antifungal prophylaxis in pediatric patients undergoing hematopoietic stem cell transplantation: a pharmacokinetic study.Biol Blood Marrow Transplant. 2010 Oct;16(10):1458-62.

Marsh RA, Madden L, Kitchen BJ, Mody R, McClimon B, Jordan MB, Bleesing JJ, Zhang K, Filipovich AH. XIAP deficiency: a unique primary immunodeficiency best classified as X-linked familial hemophagocytic lymphohistiocytosis and not as X-linked lymphoproliferative disease. Blood. 2010 Aug 19;116(7):1079-82.

Marsh RA, Satake N, Biroschak J, Jacobs T, Johnson J, Jordan MB, Bleesing JJ, Filipovich AH, Zhang K. STX11 mutations and clinical phenotypes of familial hemophagocytic lymphohistiocytosis in North America. Pediatr Blood Cancer. 2010 Jul 15;55(1):134-40.

Lykens JE, Terrell CE, Zoller EE, Divanovic S, Trompette A, Karp CL, Aliberti J, Flick MJ, Jordan MB. Mice with a selective impairment of IFN-gamma signaling in macrophage lineage cells demonstrate the critical role of IFN-gamma-activated macrophages for the control of protozoan parasitic infections in vivo. J Immunol. 2010 Jan 15;184(2):877-85.

Lin AA, Tripathi PK, Sholl A, Jordan MB, Hildeman DA. Gamma interferon signaling in macrophage lineage cells regulates central nervous system inflammation and chemokine production. J Virol. 2009 Sep;83(17):8604-15.

Marsh RA, Villanueva J, Kim MO, Zhang K, Marmer D, Risma KA, Jordan MB, Bleesing JJ, Filipovich AH. Patients with X-linked lymphoproliferative disease due to BIRC4 mutation have normal invariant natural killer T-cell populations. Clin Immunol. 2009 Jul;132(1):116-23.

Marsh RA, Villanueva J, Zhang K, Snow AL, Su HC, Madden L, Mody R, Kitchen B, Marmer D, Jordan MB, Risma KA, Filipovich AH, Bleesing JJ. A rapid flow cytometric screening test for X-linked lymphoproliferative disease due to XIAP deficiency. Cytometry B Clin Cytom. 2009 Sep;76(5):334-44.

Jordan MB, Filipovich AH. Hematopoietic cell transplantation for hemophagocytic lymphohistiocytosis: a journey of a thousand miles begins with a single (big) step. Bone Marrow Transplant. 2008 Oct;42(7):433-7.

Wojciechowski S, Jordan MB, Zhu Y, White J, Zajac AJ, Hildeman DA. Bim mediates apoptosis of CD127(lo) effector T cells and limits T cell memory. Eur J Immunol. 2006 Jul;36(7):1694-706.

Post stem cell transplant care of children; outcomes after transplant; autologous stem cell transplantation in solid tumors; allogeneic transplantation for red cell disorders; diamond blackfan anemia

MBBS, MD: University of Pune, BJ Medical College- India, 1991.

Residency: University of Illinois at Chicago, 1993-1995.

Chief Resident: University of Illinois at Chicago, 1995-1996

Fellowship: Children's Hospital Los Angeles/University of Southern California, 1996-2000.

Certification: American Board of Pediatrics, subspecialty board certified in Pediatric Hematology and Oncology, 2000 and recertified in 2007.

Loren AW, Chow E, Jacobsohn DA, Gilleece M, Halter J, Joshi S, Wang Z, Sobocinski KA, Gupta V, Hale GA, Marks DI, Stadtmauer EA, Apperley J, Cahn JY, Schouten HC, Lazarus HM, Savani BN, McCarthy PL, Jakubowski AA, Kamani NR, Hayes-Lattin B, Maziarz RT, Warwick AB, Sorror ML, Bolwell BJ, Socié G, Wingard JR, Rizzo JD, Majhail NS. Pregnancy after hematopoietic cell transplantation: a report from the late effects working committee of the Center for International Blood and Marrow Transplant Research (CIBMTR). Biol Blood Marrow Transplant. 2011 Feb;17(2):157-66. Epub 2010 Jul 24. Review.

Marsh RA, Vaughn G, Kim MO, Li D, Jodele S, Joshi S, Mehta PA, Davies SM, Jordan MB, Bleesing JJ, Filipovich AH. Reduced-intensity conditioning significantly improves survival of patients with hemophagocytic lymphohistiocytosis undergoing allogeneic hematopoietic cell transplantation. Blood. 2010 Dec 23;116(26):5824-31.

Malempati S, Joshi S, Lai S, Braner DA, Tegtmeyer K. Videos in clinical medicine. Bone marrow aspiration and biopsy. N Engl J Med. 2009 Oct 8;361(15):e28

Erdreich-Epstein A, Monforte HL, Lavey RS, Joshi S, Phillips JD, Villablanca JG.Successful multimodality therapy of recurrent multifocal juvenile granulosa cell tumor of the ovary. J Pediatr Hematol Oncol. 2002 Mar-Apr;24(3):229-33. Review.

Das A, Asatryan L, Reddy MA, Wass CA, Stins MF, Joshi S, Bonventre JV, Kwang SK. Differential role of cytosolic phospholipase A₂ in the invasion of brain microvascular endothelial cells by Escherichia coli and Listeria monocytogenes. J of Infectious Diseases. 2001184(6): 732-737.

Book Chapters

Joshi, SA, Davies SM. Hematology: Basic Principles and Practice. In Hoffmann et al (Eds.) Hematopoietic Stem Cell Transplantation for Immunodeficiencies and Genetic Diseases.

Clinical Interests

Childhood cancer and blood disorders; immune deficiency

Research Interests

Leukemia biology; cancer biology

Kumar AR, Yao Q, Li Q, Sam TA, Kersey JH. t(4;11) leukemias display addiction to MLL-AF4 but not to AF4-MLL. Leuk Res. 2011 Mar;35(3):305-9.

Kumar AR, Sarver AL, Wu B, Kersey JH. Meis1 maintains stemness signature in MLL-AF9 leukemia .Blood. 2010 Apr 29;115(17):3642-3.

Burke MJ, Cao Q, Trotz B, Weigel B, Kumar A, Smith A, Verneris MR. Allogeneic hematopoietic cell transplantation (allogeneic HCT) for treatment of pediatric Philadelphia chromosome-positive acute lymphoblastic leukemia (ALL). Pediatr Blood Cancer. 2009 Dec 15;53(7):1289-94.

Kumar AR, Li Q, Hudson WA, Chen W, Yao Q, Sam TN, Wu B, Lund EA, Kowal BJ and Kersey JH. A role for MEIS1 in MLL-fusion gene leukemia. Blood. 2009 Feb 19; 113(8):1756-8.

Chen W*, Kumar AR*, Hudson WA, Li Q, Wu B, Staggs RA, Lund EA, Sam TN and Kersey JH. Malignant transformation initiated by Mll-AF9: Gene dosage and critical target cells. Cancer Cell. 2008 May; 13: 432-440. *Co-first authors.

Kris Ann P. Schultz, MD, Joseph P. Neglia, MD, MPH, Angela R. Smith, MD, Hans D. Ochs, MD, Dr. med., Troy R. Torgerson, MD, PhD, and Ashish Kumar, MD, PhD. Familial Hemophagocytic Lymphohistiocytosis in Two Brothers With X-Linked Agammaglobulinemia.Pediatric Blood and Cancer 2008; 51:293–295.

White JG, Kumar AR, Hogan MJ. Gray Platelet Syndrome in a Somalian Family. Platelets. 2006 Dec; 17:519-527.

Mehta PA, Davies SM, Kumar A, Devidas M, Lee S, Zamzow T, Elliott J, Villanueva J, Pullen J, Zewge Y, and Filipovich A; Children’s Oncology Group. Perforin polymorphism A91V and susceptibility to B-precursor childhood acute lymphoblastic leukemia: a report from the Children's Oncology Group. Leukemia. 2006 Sep; 20; 1539-1541.

Chen W, Li Q, Hudson WA, Kumar A, Kirchhof N, Kersey JH. A murine Mll-AF4 knock-in model results in lymphoid and myeloid deregulation and hematological malignancy. Blood. 2006 Jul 15; 108:669-677.

Zhang K, Jordan MB, Marsh RA, Johnson JA, Kissell D, Meller J, Villanueva J, Risma KA, Wei Q, Klein PS, Filipovich AH. Hypomorphic mutations in PRF1, MUNC13-4, and STXBP2 are associated with adult-onset familial hemophagocytic lymphohistiocytosis. Blood. 2011 Aug 31.

Davies SM, Mehta PA. Pediatric acute lymphoblastic leukemia: is there still a role for transplant?Hematology Am Soc Hematol Educ Program. 2010;2010:363-7.

Mehta PA, Harris RE, Davies SM, Kim MO, Mueller R, Lampkin B, Mo J, Myers K, Smolarek TA. Numerical chromosomal changes and risk of development of myelodysplastic syndrome--acute myeloid leukemia in patients with Fanconi anemia. Cancer Genet Cytogenet. 2010 Dec;203(2):180-6.

Jodele S, Bleesing JJ, Mehta PA, Filipovich AH, Laskin BL, Goebel J, Pinkard SL, Davies SM. Successful early intervention for hyperacute transplant-associated thrombotic microangiopathy following pediatric hematopoietic stem cell transplantation. Pediatr Transplant. 2010 Nov 5. doi: 10.1111/j.1399-3046.2010.01408.x

Marsh RA, Vaughn G, Kim MO, Li D, Jodele S, Joshi S, Mehta PA, Davies SM, Jordan MB, Bleesing JJ, Filipovich AH. Reduced-intensity conditioning significantly improves survival of patients with hemophagocytic lymphohistiocytosis undergoing allogeneic hematopoietic cell transplantation. Blood. 2010 Dec 23;116(26):5824-31.

Laskin BL, Goebel J, Davies SM, Khoury JC, Bleesing JJ, Mehta PA, Filipovich AH, Paff ZN, Lawrence JM, Yin HJ, Pinkard SL, Jodele S. Early clinical indicators of transplant-associated thrombotic microangiopathy in pediatric neuroblastoma patients undergoing auto-SCT. Bone Marrow Transplant. 2010 Aug 9.

Mehta PA, Vinks AA, Filipovich A, Bleesing J, Jodele S, Jordan MB, Marsh R, Tarin R, Edwards S, Fearing D, Lawrence J, Davies SM. Alternate-day micafungin antifungal prophylaxis in pediatric patients undergoing hematopoietic stem cell transplantation: a pharmacokinetic study. Biol Blood Marrow Transplant. 2010 Oct;16(10):1458-62.

Mehta P, Locatelli F, Stary J, Smith FO. Bone marrow transplantation for inherited bone marrow failure syndromes. Pediatr Clin North Am. 2010 Feb;57(1):147-70. Review.

Mehta PA, Gerbing RB, Alonzo TA, Elliott JS, Zamzow TA, Combs M, Stover E, Ross JA, Perentesis JP, Meschinchi S, Lange BJ, Davies SM. FAS promoter polymorphism: outcome of childhood acute myeloid leukemia. A children's oncology group report. Clin Cancer Res. 2008 Dec 1;14(23):7896-9.

Bhatla D, Gerbing RB, Alonzo TA, Conner H, Ross JA, Meshinchi S, Zhai X, Zamzow T, Mehta PA, Geiger H, Perentesis J, Davies SM. Cytidine deaminase genotype and toxicity of cytosine arabinoside therapy in children with acute myeloid leukemia. Br J Haematol. 2009 Feb;144(3):388-94.

Mehta P, Harris R, Davies S, Kim M, Mueller R, Lampkin B, Mo J, Myers K, Smolarek T. Numerical Chromosomal Changes and Risk of Development of Myelodysplastic Syndrome/Acute Myeloid Leukemia in Patients with Fanconi Anemia. Cancer Genet Cytogenet. 2010 Dec;203(2):180-6.

Myers KC, Davies SM. Hematopoietic stem cell transplantation for bone marrow failure syndromes in children.” Biol Blood Marrow Transplant. Biol Blood Marrow Transplant. 2009 Mar;15(3):279-92. Review. 

Pozios KC, Ding J, Degger B, Upton Z, Duan C. GFs stimulate zebrafish cell proliferation by activating the MAP kinase and PI3-kinase signaling pathways. Am J Physiol Regul Integr Comp Physiol. 2001 Apr;280(4):R1230-9.

Duan C, Ding J, Li Q, Tsai W, Pozios K. Insulin-like growth factor binding protein 2 is a growth inhibitory protein conserved in zebrafish. Proc Natl Acad Sci USA. 1999 Dec 21;96(26):15274-9. 

Clinical Interests

Immune deficiency; immune dysregulation

Research Interests

Pathophysiology of perforin missense mutations identified in individuals with hemophagocytic lymphohistiocytosis; Molecular mechanisms of primary immune deficiency and dysregulation; Natural killer cell and cytotoxic T lymphocyte cytotoxicity

MD: Case Western Reserve University School of Medicine, Cleveland, OH, 1997.

PhD: Case Western Reserve University School of Medicine, Cleveland, OH, 1996.

Residency: Pediatrics, Cincinnati Children's Hospital Medical Center, 1997-2000.

Fellowship: Allergy / Immunology, Cincinnati Children's Hospital Medical Center.

Certification: Pediatrics, 2007; Allergy and Immunology, 2005.

Sumegi J, Barnes MG, Nestheide SV, Molleran-Lee S, Villanueva J, Zhang K, Risma KA, Grom AA, Filipovich AH. Gene expression profiling of peripheral blood mononuclear cells from children with active hemophagocytic lymphohistiocytosis. Blood. 2011 Apr 14;117(15):e151-60.

Uygungil B, Bleesing JJ, Risma KA, McNeal MM, Rothenberg ME. Persistent rotavirus vaccine shedding in a new case of severe combined immunodeficiency: A reason to screen. J Allergy Clin Immunol. 2010 Jan;125(1):270-1.

Marsh RA, Villanueva J, Kim MO, Zhang K, Marmer D, Risma KA, Jordan MB, Bleesing JJ, Filipovich AH. Patients with X-linked lymphoproliferative disease due to BIRC4 mutation have normal invariant natural killer T-cell populations. Clin Immunol. 2009 Jul;132(1):116-23.

Marsh RA, Villanueva J, Zhang K, Snow AL, Su HC, Madden L, Mody R, Kitchen B, Marmer D, Jordan MB, Risma KA, Filipovich AH, Bleesing JJ. A rapid flow cytometric screening test for X-linked lymphoproliferative disease due to XIAP deficiency. Cytometry B Clin Cytom. 2009 Sep;76(5):334-44.

Urrea Moreno R, Gil J, Rodriguez-Sainz C, Cela E, LaFay V, Oloizia B, Herr AB, Sumegi J, Jordan MB, Risma KA. Functional assessment of perforin C2 domain mutations illustrates the critical role for calcium-dependent lipid binding in perforin cytotoxic function. Blood. 2009 Jan 8;113(2):338-46.

Zhang K, Johnson JA, Biroschak J, Villanueva J, Lee SM, Bleesing JJ, Risma KA, Wenstrup RJ, Filipovich AH. Familial haemophagocytic lymphohistiocytosis in patients who are heterozygous for the A91V perforin variation is often associated with other genetic defects. Int J Immunogenet. 2007 Aug;34(4):231-3.

Thomas H, Risma KA, Graham TB, Brody AS, Deutsch GH, Young LR, Joseph PM. A kindred of children with interstitial lung disease. Chest. 2007 Jul;132(1):221-30.

Bullock JZ, Villanueva JM, Blanchard C, Filipovich AH, Putnam PE, Collins MH, Risma KA, Akers RM, Kirby CL, Buckmeier BK, Assa'ad AH, Hogan SP, Rothenberg ME. Interplay of adaptive th2 immunity with eotaxin-3/c-C chemokine receptor 3 in eosinophilic esophagitis. J Pediatr Gastroenterol Nutr. 2007 Jul;45(1):22-31.

Risma KA, Frayer RW, Filipovich AH, Sumegi J. Aberrant maturation of mutant perforin underlies the clinical diversity of hemophagocytic lymphohistiocytosis. J Clin Invest. 2006 Jan;116(1):182-92.

MD: University Medical School of Debrecen, Hungary, 1968.

PhD: Research Center of the Hungarian Academy of Science, Budapest, Hungary, 1974.

Docent (DMSc): Karolinska Institute Stockhom, Sweden, 1982.

Sumegi J, Barnes MG, Nestheide SV, Molleran-Lee S, Villanueva J, Zhang K, Risma KA, Grom AA, Filipovich AH. Gene expression profiling of peripheral blood mononuclear cells from children with active hemophagocytic lymphohistiocytosis. Blood. 2011 Apr 14;117(15):e151-60.

Sumegi J, Nishio J, Nelson M, Frayer RW, Perry D, Bridge JA. A novel t(4;22)(q31;q12) produces an EWSR1-SMARCA5 fusion in extraskeletal Ewing sarcoma/primitive neuroectodermal tumor. Mod Pathol. 2011 Mar;24(3):333-42.

Huang D, Sumegi J, Dal Cin P, Reith JD, Yasuda T, Nelson M, Muirhead D, Bridge JA. C11orf95-MKL2 is the resulting fusion oncogene of t(11;16)(q13;p13) in chondroid lipoma. Genes Chromosomes Cancer. 2010 Sep;49(9):810-8.

Sumegi J, Streblow R, Frayer RW, Dal Cin P, Rosenberg A, Meloni-Ehrig A, Bridge JA. Recurrent t(2;2) and t(2;8) translocations in rhabdomyosarcoma without the canonical PAX-FOXO1 fuse PAX3 to members of the nuclear receptor transcriptional coactivator family. Genes Chromosomes Cancer. 2010 Mar;49(3):224-36.

 Sumegi J, Johnson J, Filipovich A, Zhang K, Marsh R. Lymphoproliferative Disease, X-Linked. In: Pagon RA, Bird TD, Dolan CR, Stephens K, editors. GeneReviews [Internet]. Seattle (WA): University of Washington, Seattle; 1993- 2004 Feb 27 [updated 2009 Jun 18].

Trizzino A, zur Stadt U, Ueda I, Risma K, Janka G, Ishii E, Beutel K, Sumegi J, Cannella S, Pende D, Mian A, Henter JI, Griffiths G, Santoro A, Filipovich A, Aricò M; Histiocyte Society HLH Study group. Genotype-phenotype study of familial haemophagocytic lymphohistiocytosis due to perforin mutations. J Med Genet. 2008 Jan;45(1):15-21.

Erdos M, Alapi K, Balogh I, Oroszlán G, Rákóczi E, Sümegi J, Maródi L. Severe Shwachman-Diamond syndrome phenotype caused by compound heterozygous missense mutations in the SBDS gene. Exp Hematol. 2006 Nov;34(11):1517-21.

Lee SM, Sumegi J, Villanueva J, Tabata Y, Zhang K, Chakraborty R, Sheng X, Clementi R, de Saint Basile G, Filipovich AH. Patients of African ancestry with hemophagocytic lymphohistiocytosis share a common haplotype of PRF1 with a 50delT mutation. J Pediatr. 2006 Jul;149(1):134-7.

Risma KA, Frayer RW, Filipovich AH, Sumegi J. Aberrant maturation of mutant perforin underlies the clinical diversity of hemophagocytic lymphohistiocytosis. J Clin Invest. 2006 Jan;116(1):182-92.

Tabata Y, Villanueva J, Lee SM, Zhang K, Kanegane H, Miyawaki T, Sumegi J, Filipovich AH. Rapid detection of intracellular SH2D1A protein in cytotoxic lymphocytes from patients with X-linked lymphoproliferative disease and their family members. Blood. 2005 Apr 15;105(8):3066-71.

Coagulation; hemophilia; thrombosis

Gruppo RA. Treatment of hemophilia in developing countries: a journey of a thousand miles. Pediatr Blood Cancer. 2010 Mar;54(3):348-9. No abstract available

Gelfand MJ, Gruppo RA, Nasser MP. Ventilation-perfusion scintigraphy in children and adolescents is associated with a low rate of indeterminate studies. Clin Nucl Med. 2008 Sep;33(9):606-9.

Wu SW, Graham B, Gelfand MJ, Gruppo RE, Dinopolous A, Gilbert DL. Clinical and positron emission tomography findings of chorea associated with primary antiphospholipid antibody syndrome. Mov Disord. 2007 Sep 15;22(12):1813-5.

Balasa VV, Gruppo RA, Glueck CJ, Wang P, Roy DR, Wall EJ, Mehlman CT, Crawford AH. Legg-Calve-Perthes disease and thrombophilia. J Bone Joint Surg Am. 2004 Dec;86-A(12):2642-7.

Gruppo RA, Brown D, Wilkes MM, Navickis RJ. Increased breakthrough bleeding during prophylaxis with B-domain deleted factor VIII--a robust meta-analytic finding. Haemophilia. 2004 Sep;10(5):449-51.

Morrison JA, Gruppo R, Glueck CJ, Stroop D, Fontaine RN, Wang P, Smith KL. Population-specific alleles: the polymorphism (K121Q) of the human glycoprotein PC-1 gene is strongly associated with race but not with insulin resistance in black and white children. Metabolism. 2004 Apr;53(4):465-8.

Gruppo RA, Brown D, Wilkes MM, Navickis RJ. Comparative effectiveness of full-length and B-domain deleted factor VIII for prophylaxis--a meta-analysis. Haemophilia. 2003 May;9(3):251-60. Review.

Balasa VV, Kalinyak KA, Bean JA, Stroop D, Gruppo RA. Hyperhomocysteinemia is associated with low plasma pyridoxine levels in children with sickle cell disease. J Pediatr Hematol Oncol. 2002 Jun-Jul;24(5):374-9.

McNamara JL, Lombardi JP, Ferguson R, Manning PB, Gruppo RA. Alternative methods for anticoagulation monitoring in pediatric patients with applicability to a patient with severe hemophilia A and circulating inhibitor. J Extra Corpor Technol. 2001 Dec;33(4):239-42.

Gruppo R, Degrauw A, Fogelson H, Glauser T, Balasa V, Gartside P. Protein C deficiency related to valproic acid therapy: a possible association with childhood stroke. J Pediatr. 2000 Nov;137(5):714-8.

Sickle cell disease and other hemoglobinopathies; red blood cell physiology; cation transport and volume regulation; hematological problems of the newborn

Hammill AM, Risinger MA, Joiner CH, Keddache M, Kalfa TA. Compound heterozygosity for two novel mutations in the erythrocyte protein 4.2 gene causing spherocytosis in a Caucasian patient. Br J Haematol. 2011 Jan 31.

Yang MQ, Laflamme K, Gotea V, Joiner CH, Seidel NE, Wong C, Petrykowska HM, Lichtenberg J, Lee S, Welch L, Gallagher PG, Bodine DM, Elnitski L. Genome-wide detection of a TFIID localization element from an initial human disease mutation.Nucleic Acids Res. 2010 Nov 11.

George A, Pushkaran S, Li L, An X, Zheng Y, Mohandas N, Joiner CH, Kalfa TA. Altered phosphorylation of cytoskeleton proteins in sickle red blood cells: the role of protein kinase C, Rac GTPases, and reactive oxygen species. Blood Cells Mol Dis. 2010 Jun 15;45(1):41-5.

Rayburg M, Kalinyak KA, Towbin AJ, Baker PB, Joiner CH. Fatal bone marrow embolism in a child with hemoglobin SE disease.Am J Hematol. 2010 Mar;85(3):182-4.

Barber LA, Palascak MB, Joiner CH, Franco RS. Aminophospholipid translocase and phospholipid scramblase activities in sickle erythrocyte subpopulations. Br J Haematol. 2009 Aug;146(4):447-55.

Cohen RM, Franco RS, Khera PK, Smith EP, Lindsell CJ, Ciraolo PJ, Palascak MB, Joiner CH. Red cell life span heterogeneity in hematologically normal people is sufficient to alter HbA1c. Blood. 2008 Nov 15;112(10):4284-91.

Khera PK, Joiner CH, Carruthers A, Lindsell CJ, Smith EP, Franco RS, Holmes YR, Cohen RM. Evidence for interindividual heterogeneity in the glucose gradient across the human red blood cell membrane and its relationship to hemoglobin glycation.Diabetes. 2008 Sep;57(9):2445-52.

Joiner CH. Gardos pathway to sickle cell therapies? Blood. 2008 Apr 15;111(8):3918-9.

Lindsell CJ, Franco RS, Smith EP, Joiner CH, Cohen RM. A method for the continuous calculation of the age of labeled red blood cells.Am J Hematol. 2008 Jun;83(6):454-7. Erratum in: Am J Hematol. 2008 Jul;83(7):608.

Roszell NJ, Danton MJ, Jiang M, Witte D, Daugherty C, Grimes T, Girdler B, Anderson KP, Franco RS, Degen JL, Joiner CH. Fibrinogen deficiency, but not plasminogen deficiency, increases mortality synergistically in combination with sickle hemoglobin SAD in transgenic mice.Am J Hematol. 2007 Dec;82(12):1044-8.

Signaling in erythrocytes; erythropoiesis; Sickle Cell disease; endothelial cell biology

Visit the Kalfa Lab.

Hammill AM, Risinger MA, Joiner CH, Keddache M, Kalfa TA. Compound heterozygosity for two novel mutations in the erythrocyte protein 4.2 gene causing spherocytosis in a Caucasian patient. Br J Haematol. 2011 Jan 31.

Kalfa TA. Anchoring at an island to relieve stress. Blood. 2011 Jan 20;117(3):748-9.

Mizukawa B, George A, Pushkaran S, Weckbach L, Kalinyak K, Heubi JE, Kalfa TA. Cooperating G6PD mutations associated with severe neonatal hyperbilirubinemia and cholestasis.Pediatr Blood Cancer. 2010 Oct 14.

Konstantinidis DG, George A, Kalfa TA. Rac GTPases in erythroid biology. Transfus Clin Biol. 2010 Sep;17(3):126-30.

George A, Pushkaran S, Li L, An X, Zheng Y, Mohandas N, Joiner CH, Kalfa TA. Altered phosphorylation of cytoskeleton proteins in sickle red blood cells: the role of protein kinase C, Rac GTPases, and reactive oxygen species. Blood Cells Mol Dis. 2010 Jun 15;45(1):41-5.

Kalfa TA, Pushkaran S, Zhang X, Johnson JF, Pan D, Daria D, Geiger H, Cancelas JA, Williams DA, Zheng Y. Rac1 and Rac2 GTPases are necessary for early erythropoietic expansion in the bone marrow but not in the spleen.Haematologica. 2010 Jan;95(1):27-35.

Mulloy JC, Cancelas JA, Filippi MD, Kalfa TA, Guo F, Zheng Y. Rho GTPases in hematopoiesis and hemopathies. Blood. 2010 Feb 4;115(5):936-47.

Wang D, Zhang W, Kalfa TA, Grabowski G, Davies S, Malik P, Pan D. Reprogramming erythroid cells for lysosomal enzyme production leads to visceral and CNS cross-correction in mice with Hurler syndrome.Proc Natl Acad Sci U S A. 2009 Nov 24;106(47):19958-63.

Diwan A, Koesters AG, Capella D, Geiger H, Kalfa TA, Dorn GW 2nd. Targeting erythroblast-specific apoptosis in experimental anemia. Apoptosis. 2008 Aug;13(8):1022-30.

Daria D, Filippi MD, Knudsen ES, Faccio R, Li Z, Kalfa T, Geiger H. The retinoblastoma tumor suppressor is a critical intrinsic regulator for hematopoietic stem and progenitor cells under stress. Blood. 2008 Feb 15;111(4):1894-902.

Diwan A, Koesters AG, Odley AM, Pushkaran S, Baines CP, Spike BT, Daria D, Jegga AG, Geiger H, Aronow BJ, Molkentin JD, Macleod KF, Kalfa TA, Dorn GW. Unrestrained erythroblast development in Nix-/- mice reveals a mechanism for apoptotic modulation of erythropoiesis. Proc Natl Acad Sci U S A. 2007 Apr 17;104(16):6794-9.

Kalfa TA, Pushkaran S, Mohandas N, Hartwig JH, Fowler VM, Johnson JF, Joiner CH, Williams DA, Zheng Y. Rac GTPases regulate the morphology and deformability of the erythrocyte cytoskeleton. Blood. 2006 Dec 1;108(12):3637-45.

Hematology; bone marrow failure; sickle cell anemia; hemoglobinopathy

Wang W, Brugnara C, Snyder C, Wynn L, Rogers Z, Kalinyak K, Brown C, Qureshi A, Bigelow C, Neumayr L, Smith-Whitley K, Chui DH, Delahunty M, Woolson R, Steinberg M, Telen M, Kesler K. The effects of hydroxycarbamide and magnesium on haemoglobin SC disease: results of the multi-centre CHAMPS trial.Br J Haematol. 2011 Jan 31.

Mizukawa B, George A, Pushkaran S, Weckbach L, Kalinyak K, Heubi JE, Kalfa TA. Cooperating G6PD mutations associated with severe neonatal hyperbilirubinemia and cholestasis.Pediatr Blood Cancer. 2010 Oct 14.

Rayburg M, Kalinyak KA, Towbin AJ, Baker PB, Joiner CH. Fatal bone marrow embolism in a child with hemoglobin SE disease.Am J Hematol. 2010 Mar;85(3):182-4.

Crosby LE, Modi AC, Lemanek KL, Guilfoyle SM, Kalinyak KA, Mitchell MJ. Perceived barriers to clinic appointments for adolescents with sickle cell disease. J Pediatr Hematol Oncol. 2009 Aug;31(8):571-6.

Grueneich R, Ris MD, Ball W, Kalinyak KA, Noll R, Vannatta K, Wells R. Relationship of structural magnetic resonance imaging, magnetic resonance perfusion, and other disease factors to neuropsychological outcome in sickle cell disease.J Pediatr Psychol. 2004 Mar;29(2):83-92.

Koontz K, Short AD, Kalinyak K, Noll RB. A randomized, controlled pilot trial of a school intervention for children with sickle cell anemia.J Pediatr Psychol. 2004 Jan-Feb;29(1):7-17.

Balasa VV, Kalinyak KA, Bean JA, Stroop D, Gruppo RA. Hyperhomocysteinemia is associated with low plasma pyridoxine levels in children with sickle cell disease.J Pediatr Hematol Oncol. 2002 Jun-Jul;24(5):374-9.

Graumlich SE, Powers SW, Byars KC, Schwarber LA, Mitchell MJ, Kalinyak KA. Multidimensional assessment of pain in pediatric sickle cell disease. J Pediatr Psychol. 2001 Jun;26(4):203-14.

Noll RB, Stith L, Gartstein MA, Ris MD, Grueneich R, Vannatta K, Kalinyak K. Neuropsychological functioning of youths with sickle cell disease: comparison with non-chronically ill peers.J Pediatr Psychol. 2001 Mar;26(2):69-78.

Balasa VV, Gruppo RA, Gartside PS, Kalinyak KA. Correlation of the C677T MTHFR genotype with homocysteine levels in children with sickle cell disease.J Pediatr Hematol Oncol. 1999 Sep-Oct;21(5):397-400.

Clinical Interests

Clinical hematology / oncology; hematopathology

Research Interests

Leukemia; solid tumors; chemotherapeutic agents

Mehta PA, Harris RE, Davies SM, Kim MO, Mueller R, Lampkin B, Mo J, Myers K, Smolarek TA. Numerical chromosomal changes and risk of development of myelodysplastic syndrome--acute myeloid leukemia in patients with Fanconi anemia. Cancer Genet Cytogenet. 2010 Dec;203(2):180-6.

Mo J, Lampkin B, Perentesis J, Poole L, Bao L. Translocation (8;18;16)(p11;q21;p13). A new variant of t(8;16)(p11;p13) in acute monoblastic leukemia: case report and review of the literature.Cancer Genet Cytogenet. 2006 Feb;165(1):75-8. Review.

Wells RJ, Arthur DC, Srivastava A, Heerema NA, Le Beau M, Alonzo TA, Buxton AB, Woods WG, Howells WB, Benjamin DR, Betcher DL, Buckley JD, Feig SA, Kim T, Odom LF, Ruymann FB, Smithson WA, Tannous R, Whitt JK, Wolff L, Tjoa T, Lampkin BC. Prognostic variables in newly diagnosed children and adolescents with acute myeloid leukemia: Children's Cancer Group Study 213.Leukemia. 2002 Apr;16(4):601-7.

Sande JE, Arceci RJ, Lampkin BC. Congenital and neonatal leukemia. Semin Perinatol. 1999 Aug;23(4):274-85. Review.

Mazewski C, Soukup S, Ballard E, Gotwals B, Lampkin B. Karyotype studies in 18 ependymomas with literature review of 107 cases. Cancer Genet Cytogenet. 1999 Aug;113(1):1-8. Review.

Arceci RJ, Reaman GH, Cohen AR, Lampkin BC. Position statement for the need to define pediatric hematology/oncology programs: a model of subspecialty care for chronic childhood diseases. Health Care Policy and Public Issues Committee of the American Society of Pediatric Hematology/Oncology.J Pediatr Hematol Oncol. 1998 Mar-Apr;20(2):98-103.

Klopfenstein K, Soukup S, Blough R, Mazewski C, Ballard E, Gotwals B, Lampkin B. Chromosome analyses in a rhabdoid tumor of the brain.Cancer Genet Cytogenet. 1997 Feb;93(2):152-6.

Miller CW, Aslo A, Won A, Tan M, Lampkin B, Koeffler HP. Alterations of the p53, Rb and MDM2 genes in osteosarcoma.J Cancer Res Clin Oncol. 1996;122(9):559-65.

George BA, Yanik G, Wells RJ, Martin LW, Soukup S, Ballard ET, Gartside PS, Lampkin BC. Growth patterns of human neuroblastoma xenografts and their relationship to treatment outcome.Cancer. 1993 Dec 1;72(11):3331-9.

Kadam P, Umerani A, Srivastava A, Masterson M, Lampkin B, Raza A. Combination of classical and interphase cytogenetics to investigate the biology of myeloid disorders: detection of masked monosomy 7 in AML.Leuk Res. 1993 Apr;17(4):365-74.

Arumugam P, Malik P. Genetic therapy for beta-thalassemia: from the bench to the bedside.Hematology Am Soc Hematol Educ Program. 2010;2010:445-50.

Perumbeti A, Malik P. Therapy for beta-globinopathies: a brief review and determinants for successful and safe correction.Ann N Y Acad Sci. 2010 Aug;1202:36-44. Review.

Perumbeti A, Malik P. Genetic correction of sickle cell anemia and beta-thalassemia: progress and new perspective.ScientificWorldJournal. 2010 Apr 13;10:644-54. Review.

Sundaram N, Tailor A, Mendelsohn L, Wansapura J, Wang X, Higashimoto T, Pauciulo MW, Gottliebson W, Kalra VK, Nichols WC, Kato GJ, Malik P. High levels of placenta growth factor in sickle cell disease promote pulmonary hypertension.Blood. 2010 Jul 8;116(1):109-12.

Wang D, Zhang W, Kalfa TA, Grabowski G, Davies S, Malik P, Pan D. Reprogramming erythroid cells for lysosomal enzyme production leads to visceral and CNS cross-correction in mice with Hurler syndrome.Proc Natl Acad Sci U S A. 2009 Nov 24;106(47):19958-63.

Arumugam PI, Urbinati F, Velu CS, Higashimoto T, Grimes HL, Malik P. The 3' region of the chicken hypersensitive site-4 insulator has properties similar to its core and is required for full insulator activity.PLoS One. 2009 Sep 10;4(9):e6995.

Arumugam PI, Higashimoto T, Urbinati F, Modlich U, Nestheide S, Xia P, Fox C, Corsinotti A, Baum C, Malik P. Genotoxic potential of lineage-specific lentivirus vectors carrying the beta-globin locus control region.Mol Ther. 2009 Nov;17(11):1929-37.

Perumbeti A, Higashimoto T, Urbinati F, Franco R, Meiselman HJ, Witte D, Malik P. A novel human gamma-globin gene vector for genetic correction of sickle cell anemia in a humanized sickle mouse model: critical determinants for successful correction.Blood. 2009 Aug 6;114(6):1174-85.

Urbinati F, Arumugam P, Higashimoto T, Perumbeti A, Mitts K, Xia P, Malik P. Mechanism of reduction in titers from lentivirus vectors carrying large inserts in the 3'LTR.Mol Ther. 2009 Sep;17(9):1527-36.

Williams JP, Wu J, Johansson G, Rizvi TA, Miller SC, Geiger H, Malik P, Li W, Mukouyama YS, Cancelas JA, Ratner N. Nf1 mutation expands an EGFR-dependent peripheral nerve progenitor that confers neurofibroma tumorigenic potential.Cell Stem Cell. 2008 Dec 4;3(6):658-69.

Interactions between the hemostatic system and innate immunity effecting tumor progression; Langerhans' cell hystiocytosis

Horowitz NA, Blevins EA, Miller WM, Perry AR, Talmage KE, Mullins ES, Flick MJ, Queiroz KC, Shi K, Spek CA, Conway EM, Monia BP, Weiler H, Degen JL, Palumbo JS. Thrombomodulin is a determinant of metastasis through a mechanism linked to the thrombin binding domain but not the lectin-like domain. Blood. 2011 Jul 25.

Flick MJ, Chauhan AK, Frederick M, Talmage KE, Kombrinck KW, Miller W, Mullins ES, Palumbo JS, Zheng X, Esmon NL, Esmon CT, Thornton S, Becker A, Pelc LA, Di Cera E, Wagner DD, Degen JL. The development of inflammatory joint disease is attenuated in mice expressing the anticoagulant prothrombin mutant W215A/E217A. Blood. 2011 Jun 9;117(23):6326-37.

Akunuru S, Palumbo J, Zhai QJ, Zheng Y. Rac1 targeting suppresses human non-small cell lung adenocarcinoma cancer stem cell activity. PLoS One. 2011 Feb 9;6(2):e16951.

Palumbo JS, Degen JL. Mechanisms coupling the hemostatic system to colitis-associated cancer. Thromb Res. 2010 Apr;125 Suppl 2:S39-43. Review.

Steinbrecher KA, Horowitz NA, Blevins EA, Barney KA, Shaw MA, Harmel-Laws E, Finkelman FD, Flick MJ, Pinkerton MD, Talmage KE, Kombrinck KW, Witte DP, Palumbo JS. Colitis-associated cancer is dependent on the interplay between the hemostatic and inflammatory systems and supported by integrin alpha(M)beta(2) engagement of fibrinogen.Cancer Res. 2010 Apr 1;70(7):2634-43.

Palumbo JS. Mechanisms linking tumor cell-associated procoagulant function to tumor dissemination.Semin Thromb Hemost. 2008 Mar;34(2):154-60. Review.

Palumbo JS, Barney KA, Blevins EA, Shaw MA, Mishra A, Flick MJ, Kombrinck KW, Talmage KE, Souri M, Ichinose A, Degen JL. Factor XIII transglutaminase supports hematogenous tumor cell metastasis through a mechanism dependent on natural killer cell function.J Thromb Haemost. 2008 May;6(5):812-9.

Palumbo JS, Degen JL. Mechanisms linking tumor cell-associated procoagulant function to tumor metastasis.Thromb Res. 2007;120 Suppl 2:S22-8.

Flick MJ, LaJeunesse CM, Talmage KE, Witte DP, Palumbo JS, Pinkerton MD, Thornton S, Degen JL. Fibrin(ogen) exacerbates inflammatory joint disease through a mechanism linked to the integrin alphaMbeta2 binding motif.J Clin Invest. 2007 Nov;117(11):3224-35.

Palumbo JS, Talmage KE, Massari JV, La Jeunesse CM, Flick MJ, Kombrinck KW, Hu Z, Barney KA, Degen JL. Tumor cell-associated tissue factor and circulating hemostatic factors cooperate to increase metastatic potential through natural killer cell-dependent and-independent mechanisms.Blood. 2007 Jul 1;110(1):133-41.

Clinical Interests

Sickle cell disease; hemoglobinopathies

Research Interests

Gene therapy strategies for sickle cell disease

Perumbeti A, Malik P. Therapy for beta-globinopathies: a brief review and determinants for successful and safe correction.Ann N Y Acad Sci. 2010 Aug;1202:36-44. Review.

Perumbeti A, Malik P. Genetic correction of sickle cell anemia and beta-thalassemia: progress and new perspective.ScientificWorldJournal. 2010 Apr 13;10:644-54. Review.

Perumbeti A, Higashimoto T, Urbinati F, Franco R, Meiselman HJ, Witte D, Malik P. A novel human gamma-globin gene vector for genetic correction of sickle cell anemia in a humanized sickle mouse model: critical determinants for successful correction.Blood. 2009 Aug 6;114(6):1174-85.

Urbinati F, Arumugam P, Higashimoto T, Perumbeti A, Mitts K, Xia P, Malik P. Mechanism of reduction in titers from lentivirus vectors carrying large inserts in the 3'LTR.Mol Ther. 2009 Sep;17(9):1527-36.

Clinical Interests

Sickle cell disease; thalassemia; hemoglobinopathies; disorders of red blood cells; iron overload and iron chelation; chronic transfusion therapy

Research Interests

Novel causes of sickle cell pain; Role of hemoglobin desaturation in the pathogenesis of sickle cell disease; Survival and long-term follow-up of sickle cell disease; Causes and treatment of stroke in sickle cell disease

MD:  University of Texas Southwestern Medical Center, Dallas, TX, 1994.

MS:  University of Texas Southwestern Medical Center, Dallas, TX, 2008.

Residency & Chief Residency:  Children's Medical Center Dallas, Dallas, TX; University of Texas Southwestern Medical Center, Dallas, TX, 1998.

Fellowship:  Children's Medical Center Dallas, Dallas, TX; University of Texas Southwestern Medical Center, Dallas, TX, 2001.

Certifications:  Pediatrics, 1998; Pediatric Hematology-Oncology, 2002.

Quinn CT, Johnson VL, Kim HY, Trachtenberg F, Vogiatzi MG, Kwiatkowski JL, Neufeld EJ, Fung E, Oliveri N, Kirby M, Giardina PJ; for the Thalassemia Clinical Research Network. Renal dysfunction in patients with thalassaemia. Br J Haematol. 2011 Feb 21. doi: 10.1111/j.1365-2141.2010.08477.x.

McCavit TL, Quinn CT, Techasaensiri C, Rogers ZR. Increase in Invasive Streptococcus Pneumoniae Infections in Children with Sickle Cell Disease since Pneumococcal Conjugate Vaccine Licensure. J Pediatr. 2011 Mar;158(3):505-7.

Sobota A, Yamashita R, Xu Y, Trachtenberg F, Kohlbry P, Kleinert DA, Giardina PJ, Kwiatkowski JL, Foote D, Thayalasuthan V, Porter JB, Thompson AA, Schilling L, Quinn CT, Neufeld EJ; Thalassemia Clinical Research Network. Quality of life in thalassemia: a comparison of SF-36 results from the thalassemia longitudinal cohort to reported literature and the US norms. Am J Hematol. 2011 Jan;86(1):92-5.

Mednick L, Yu S, Trachtenberg F, Xu Y, Kleinert DA, Giardina PJ, Kwiatkowski JL, Foote D, Thayalasuthan V, Porter JB, Thompson AA, Schilling L, Quinn CT, Neufeld EJ, Yamashita R; Thalassemia Clinical Research Network. Symptoms of depression and anxiety in patients with thalassemia: prevalence and correlates in the thalassemia longitudinal cohort. Am J Hematol. 2010 Oct;85(10):802-5.

Jordan LC, McKinstry RC 3rd, Kraut MA, Ball WS, Vendt BA, Casella JF, DeBaun MR, Strouse JJ; Silent Infarct Transfusion Trial Investigators. Incidental findings on brain magnetic resonance imaging of children with sickle cell disease. Pediatrics. 2010 Jul;126(1):53-61.

Fung EB, Xu Y, Kwiatkowski JL, Vogiatzi MG, Neufeld E, Olivieri N, Vichinsky EP, Giardina PJ; Thalassemia Clinical Research Network. Relationship between chronic transfusion therapy and body composition in subjects with thalassemia. J Pediatr. 2010 Oct;157(4):641-7, 647.e1-2.

Quinn CT, Rogers ZR, McCavit TL, Buchanan GR. Improved survival of children and adolescents with sickle cell disease. Blood. 2010 Apr 29;115(17):3447-52.

Dowling MM, Lee N, Quinn CT, Rogers ZR, Boger D, Ahmad N, Ramaciotti C, Buchanan GR. Prevalence of intracardiac shunting in children with sickle cell disease and stroke. J Pediatr. 2010 Apr;156(4):645-50.

Ghatpande SS, Choudhary PK, Quinn CT, Goodman SR. In vivo pharmaco-proteomic analysis of hydroxyurea induced changes in the sickle red blood cell membrane proteome. J Proteomics. 2010 Jan 3;73(3):619-26.

Dowling MM, Quinn CT, Rogers ZR, Buchanan GR. Acute silent cerebral infarction in children with sickle cell anemia. Pediatr Blood Cancer. 2010 Mar;54(3):461-4.

MD: University of Rochester School of Medicine and Dentistry, Rochester, NY, 1989.

Residency: Pediatrics, Children's Hospital, Boston, MA.

Fellowship: Neurology, Children's Hospital, Boston, MA.

Certification: Pediatrics, 1996; Neurology, 1996.

MD: Stanford University School of Medicine, Stanford, CA, 2003.

Residency: Yale-New Haven Hospital, New Haven, CT, 2006.

Fellowship: University of Texas Southwestern Medical Center Dallas, Dallas, TX, 2009.

Certification: Pediatrics, 2006.
 

Relapsed leukemia; lymphoma; new therapeutics; ataxia telangiectasia; DNA damage response mechanisms

BS: Lewis and Clark College, Portland, OR, 1987.

PhD: Massachusetts Institute of Technology, Cambridge, MA, 1994.

MD: Oregon Health Sciences University, Portland, OR, 1998.

Fellowship: St. Jude Children's Research Hospital, Memphis, TN, 2005.

Absalon MJ, Smith FO. Treatment strategies for pediatric acute myeloid leukemia. Expert Opin Pharmacother. 2009 Jan;10(1):57-79.

Absalon MJ, McCarville MB, Liu T, Santana VM, Daw NC, Navid F. Pulmonary nodules discovered during the initial evaluation of pediatric patients with bone and soft-tissue sarcoma. Pediatr Blood Cancer. 2008 Jun;50(6):1147-53.

Takagi M, Absalon MJ, McLure KG, Kastan MB. Regulation of p53 translation and induction after DNA damage by ribosomal protein L26 and nucleolin. Cell. 2005 Oct 7;123(1):49-63.

Absalon MJ, Harding CO, Fain DR, Li L, Mack KJ. Leigh syndrome in an infant due to mitochondrial DNA depletion.  Pediatr Neurology. 2001; 24:60-63.

Fadell MF 2nd, Jones BV, Adams DM. Prenatal diagnosis and postnatal follow-up of rapidly involuting congenital hemangioma (RICH). Pediatr Radiol. 2011 Aug;41(8):1057-60.

Maugans T, Sheridan RM, Adams D, Gupta A. Cutaneous vascular anomalies associated with neural tube defects: nomenclature and pathology revisited. Neurosurgery. 2011 Jul;69(1):112-8.

Hammill AM, Wentzel M, Gupta A, Nelson S, Lucky A, Elluru R, Dasgupta R, Azizkhan RG, Adams DM. Sirolimus for the treatment of complicated vascular anomalies in children. Pediatr Blood Cancer. 2011 Mar 28. Epub ahead of print.

Adams DM. Special considerations in vascular anomalies: hematologic management. Clin Plast Surg. 2011 Jan;38(1):153-60.

Duffy KJ, Runge-Samuelson C, Bayer ML, Friedland D, Sulman C, Chun R, Kerschner JE, Metry D, Adams D, Drolet BA. Association of hearing loss with PHACE syndrome. Arch Dermatol. 2010 Dec;146(12):1391-6.

Drolet BA, Chamlin SL, Garzon MC, Adams D, Baselga E, Haggstrom AN, Holland KE, Horii KA, Juern A, Lucky AW, Mancini AJ, McCuaig C, Metry DW, Morel KD, Newell BD, Nopper AJ, Powell J, Frieden IJ. Prospective study of spinal anomalies in children with infantile hemangiomas of the lumbosacral skin. J Pediatr. 2010 Nov;157(5):789-94.

Roganović J, Adams D. PHACES syndrome--case report and literature review. Coll Antropol. 2009 Mar;33(1):311-4.

Lomenick JP, Reifschneider KL, Lucky AW, Adams D, Azizkhan RG, Woo JG, Backeljauw PF. Prevalence of adrenal insufficiency following systemic glucocorticoid therapy in infants with hemangiomas. Arch Dermatol. 2009 Mar;145(3):262-6.

Dickie B, Dasgupta R, Nair R, Alonso MH, Ryckman FC, Tiao GM, Adams DM, Azizkhan RG. Spectrum of hepatic hemangiomas: management and outcome. J Pediatr Surg. 2009 Jan;44(1):125-33.

Adams DM. 50 Years Ago in the Journal of Pediatrics. Hemangioma with Thrombocytopenia. J Pediatrics. 2009;154(4):497.

Burns K, Broudreau C, Panepinto J. Attitudes Regarding Fertility in Adolescent Females Diagnosed with Cancer. J Pediatr Hematol Oncol. 2006 Jun;28(6): 350-354.

Burns K and Camitta B. Pyrite or True Gold? Journal of Pediatric Hematology/Oncology. 2005 May;27(5): 244.

Clinical Interests

Pediatric neuro-oncology

Research Interests

Mouse models for glioma; molecular profiling of tumor mutations; biomarkers of tumor progression; novel therapeutic agents for glioma

Visit the Chow Lab

Chow LML, Endersby R, Zhu X, Rankin S, Qu C, Zhang J, Broniscer A, Ellison DW, Baker SJ. Cooperativity within and among Pten, p53 and Rb pathways induces high-grade astrocytoma in adult brain. Cancer Cell. 2011;19:305-316.

Lavado A, Lagutin O, Chow LML, Baker SJ, Oliver G. Prox1 is required for granule cell maturation and intermediate progenitor maintenance during brain neurogenesis. PLoS Biol. 2010;8:e1000460.

Cicero SA, Johnson D, Reyntjens S, Frase S, Connell S, Chow LML, Baker SJ, Sorrentino BP, Dyer MA. Cells previously identified as retinal stem cells are pigmented ciliary epithelial cells. Proc Natl Acad Sci U S A. 2009 Apr;106(16):6685-90.

Weber T, Corbett MK, Chow LML, Valentine MB, Baker SJ, Zuo J. Rapid cell-cycle reentry and cell death after acute inactivation of the retinoblastoma gene product in postnatal cochlear hair cells. Proc Natl Acad Sci U S A. 2008;105(2):781-5.

Chow LML, Zhang J, Baker SJ. Inducible Cre recombinase activity in mouse mature astrocytes and adult neural precursor cells. Transgenic Res. 2008;17(5):919-28. 

Chow LM, Nathan PC, Hodgson DC, Jenkin D, Weitzman S, Grant RM, Manson D, Bross A, Doyle JJ, Danjoux C, Greenberg ML. Survival and late effects in children with Hodgkin’s lymphoma treated with MOPP/ABV and low-dose, extended-field irradiation. J Clin Oncol. 2006;24:5735-5741 

Chow LM, Tian Y, Weber T, Corbett M, Zuo J, Baker SJ. Inducible Cre recombinase activity in mouse cerebellar granule cell precursors and inner ear hair cells. Dev Dyn. 2006;235:2991-2998.

Chow LML, Baker SJ. PTEN function in normal and neoplastic growth. Cancer Lett. 2006;241:184-196. 

Pediatric cancers; leukemia and lymphoma; new drug development

MD: Emory University School of Medicine, Atlanta, GA, 2005.

Residency: Pediatrics, University of Texas Southwestern Medical Center, Dallas, TX.

Fellowship: Pediatric Hematology/Oncology, University of Texas Southwestern Medical Center, Dallas, TX.

Certification: Pediatrics, 2008.

Dasgupta B, Milbrandt J. AMP-activated protein kinase phosphorylates retinoblastoma protein to control mammalian brain development. Dev Cell. 2009 Feb;16(2):256-70.

Dasgupta B, Milbrandt J. Resveratrol stimulates AMP kinase activity in neurons. Proc Natl Acad Sci U S A. 2007 Apr;24;104(17):7217-22.

Revollo JR, Körner A, Mills KF, Satoh A, Wang T, Garten A, Dasgupta B, Sasaki Y, Wolberger C, Townsend RR, Milbrandt J, Kiess W, Imai S. Nampt/PBEF/Visfatin regulates insulin secretion in beta cells as a systemic NAD biosynthetic enzyme. Cell Metab. 2007 Nov;6(5):363-75.

Hegedus B, Dasgupta B, Shin JE, Emnett RJ, Hart-Mahon EK, Elghazi L, Bernal-Mizrachi E, Gutmann DH. Neurofibromatosis-1 regulates neuronal and glial cell differentiation from neuroglial progenitors in vivo by both cAMP- and Ras-dependent mechanisms. Cell Stem Cell. 2007 Oct 11;1(4):443-57.

Warrington NM, Woerner BM, Daginakatte GC, Dasgupta B, Perry A, Gutmann DH, Rubin JB. Spatiotemporal differences in CXCL12 expression and cyclic AMP underlie the unique pattern of optic glioma growth in neurofibromatosis type 1. Cancer Res. 2007 Sep 15;67(18):8588-95.

Dasgupta B, Gutmann DH. Neurofibromin regulates neural stem cell proliferation, survival, and astroglial differentiation in vitro and in vivo. J Neurosci. 2005 Jun 8;25(23):5584-94.

Dasgupta B, Yi Y, Chen DY, Weber JD, Gutmann DH. Proteomic analysis reveals hyperactivation of the mammalian target of rapamycin pathway in neurofibromatosis 1-associated human and mouse brain tumors. Cancer Res. 2005 Apr 1;65(7):2755-60.

Dasgupta B, Yi Y, Hegedus B, Weber JD, Gutmann DH. Cerebrospinal fluid proteomic analysis reveals dysregulation of methionine aminopeptidase-2 expression in human and mouse neurofibromatosis 1-associated glioma. Cancer Res. 2005 Nov 1;65(21):9843-50.

Dasgupta B, Li W, Perry A, Gutmann DH. Glioma formation in neurofibromatosis 1 reflects preferential activation of K-RAS in astrocytes. Cancer Res. 2005 Jan 1;65(1):236-45.

Dasgupta B, Dugan LL, Gutmann DH. The neurofibromatosis 1 gene product neurofibromin regulates pituitary adenylate cyclase-activating polypeptide-mediated signaling in astrocytes. J Neurosci. 2003 Oct 1;23(26):8949-54.

Dasgupta B, Roychoudhury K, Ganguly S, Akbar MA, Das P, Roy S. Infection of human mononuclear phagocytes and macrophage-like THP1 cells with Leishmania donovani results in modulation of expression of a subset of chemokines and a chemokine receptor. Scand J Immunol. 2003 Apr;57(4):366-74.

BS: Human Biology, University of Toronto, Toronto, Canada, 1987. 

MD: University of Toronto, Toronto, Canada, 1991.

MSc: Institute of Medical Science, University of Toronto, Toronto, Canada, 2002.

Dorris K, Fouladi M, Davies SM, Perentesis JP, Lawrence JM, Chow LM, Assa'ad A, Uygungil B, Jodele S. Severe Allergic Reactions to Thiol-based Cytoprotective Agents Mesna and Amifostine in a Child With a Supratentorial Primitive Neuroectodermal Tumor. J Pediatr Hematol Oncol. 2011 Aug;33(6):e250-2.

Phillips CL, Miles L, Jones BV, Sutton M, Crone K, Fouladi M. Medulloblastoma with melanotic differentiation: case report and review of the literature. J Neurooncol. 2011 Jul;103(3):759-64.

Fouladi M, Gururangan S, Moghrabi A, Phillips P, Gronewold L, Wallace D, Sanford RA, Gajjar A, Kun LE, Heideman R. Carboplatin-based primary chemotherapy for infants and young children with CNS tumors. Cancer. 2009 Jul 15;115(14):3243-53.

Shih C, Hale GA, Gronewold L, Tong X, Gilger EA, Srivastava DK, Kun LE, Gajjar A, Fouladi M. High-Dose Chemotherapy with Autologous Stem Cell Rescue for Children with Recurrent Malignant Brain Tumors. Cancer. Mar 15;112(6):1345-53.

Fouladi M, Nicholson S, Zhou T, Laningham F, Helton K, et al. A Phase II Study of the Farnesyl Transferase Inhibitor, Tipifarnib, in Children with Recurrent or Progressive High Grade Glioma, Medulloblastoma/PNET or Brainstem Glioma: A Children’s Oncology Group Study. Cancer. 2007 Dec 1;110(11):2535-41. 

Fouladi M, Laningham F, Wu J, O’Shaughnessy M, Molina K, Broniscer A, Spunt SL, Stewart CF, Houghton PJ, Gilbertson RJ, Furman WL. Phase I Study of Everolimus (RAD001) in Pediatric Patients with Refractory Solid Tumors. J Clin Oncol. 2007 Oct 20;25(30):4806-12.

Morris B, Gajjar A, Okuma, J, Yutaka Y, Wallace D, Kun L, Merchant T, Fouladi M, Broniscer A, Robison L, Hudson M. Survival and Late Mortality in Long-term Survivors of Pediatric Central Nervous System Tumors. J Clin Oncol. 2007 Apr 20;25(12):1532-8.

Gajjar A, Chintagumpala M, Ashley D, Kellie S, Kun LE, Merchant TE, Woo S, Wheeler G, Ahern V, Krasin MJ, Fouladi M, Broniscer A, Krance R, Hale GA, Stewart CF, Dauser R, Sanford RA, Fuller C, Lau C, Boyett JM, Wallace D, Gilbertson RJ. Risk-adapted craniospinal radiotherapy followed by high-dose chemotherapy and stem-cell rescue in children with newly diagnosed medulloblastoma (St Jude Medulloblastoma-96): long-term results from a prospective, multicenter trial.Lancet Oncol. 2006 Oct;7(10):813-20. Erratum in: Lancet Oncol. 2006 Oct;7(10):797.

Fouladi M, Blaney S, Young Poussaint T, Freeman B, McLendon R, Fuller C, Adesina A, Hancock M, Danks M, Ivy P, Stewart C, Gajjar A. A Phase II study of oxaliplatin in children with recurrent or refactory medulloblastoma (MB), supratentorial primitive neuroectodermal tumors (SPNET) and atypical teratoid rhabdoid tumors (ATRT): A Pediatric Brain Tumor Consortium Study. Cancer. 2006 107:2291-2297.

Fouladi M. Histone deacetylase inhibitors. Cancer Invest. 2006 (5):521-7.

Merchant T, Fouladi M. Ependymoma: New therapeutic approaches including radiation and chemotherapy. J Neuro Oncol. 2006 3:287-99.

Clinical Interests

Developmental therapeutics; renal / liver / retinoblastoma / neuro-oncology

Research Interests

Elucidating ways to translate the use of biological response modifiers in combination with conventional chemotherapeutics

MD: Sackler School of Medicine, 1997.

Residency: New York Medical College, 2000.

Fellowship: St Jude Children's Research Hospital, 2004.

Certification: Pediatrics, 2000, 2007; Pediatric Hematology / Oncology, 2005.

Geller JI, Meyers AB, Towbin AJ, Serai S, Geller JI, Podberesky DJ. Characterization of pediatric liver lesions with gadoxetate disodium. Pediatr Radiol. 2011 Sep;41(9):1183-97.

Pressey JG, Wright JM, Geller JI, Joseph DB, Pressey CS, Kelly DR. Sirolimus therapy for fibromatosis and multifocal renal cell carcinoma in a child with tuberous sclerosis complex. Pediatr Blood Cancer. 2010 Jul 1;54(7):1035-7.

Cripe TP. Adenovirus gene therapy for pediatric cancers: shall we gather at the liver? Pediatr Blood Cancer. 2009 Aug;53(2):133-5.

Geller JI, Dome JS. Retroperitoneal lymph node dissection for pediatric renal cell carcinoma. Pediatr Blood Cancer. 2009 Mar;52(3):430.

Geller JI, Leslie ND, Yin H. Malignant Rhabdoid Tumor. eMedicine from WebMD. 2009 Dec. Available online.

Geller JI, Wall D, Perentesis J, Blaney SM, Bernstein M; Pediatric Oncology Group study 9376. Phase I study of paclitaxel with standard dose ifosfamide in children with refractory solid tumors: a Pediatric Oncology Group study (POG 9376). Pediatr Blood Cancer. 2009 Mar;52(3):346-50.

Geller JI. Genetic stratification of Wilms tumor: is WT1 gene analysis ready for prime time?Cancer. 2008 Sep 1;113(5):893-6.

Geller JI, Argani P, Adeniran A, Hampton E, De Marzo A, Hicks J, Collins MH. Translocation renal cell carcinoma: lack of negative impact due to lymph node spread. Cancer. 2008 Apr 1;112(7):1607-16.

Geller JI, Dome JS. Adjuvant therapy in pediatric patients with completely resected renal cell carcinoma. Pediatr Blood Cancer. 2006 Apr;46(4):527.

Geller JI, Dome JS. Local lymph node involvement does not predict poor outcome in pediatric renal cell carcinoma. Cancer. 2004 Oct 1;101(7):1575-83.

Clinical Interests

Clinical hematology / oncology; hematopathology

Research Interests

Leukemia; solid tumors; chemotherapeutic agents

Mehta PA, Harris RE, Davies SM, Kim MO, Mueller R, Lampkin B, Mo J, Myers K, Smolarek TA. Numerical chromosomal changes and risk of development of myelodysplastic syndrome--acute myeloid leukemia in patients with Fanconi anemia. Cancer Genet Cytogenet. 2010 Dec;203(2):180-6.

Mo J, Lampkin B, Perentesis J, Poole L, Bao L. Translocation (8;18;16)(p11;q21;p13). A new variant of t(8;16)(p11;p13) in acute monoblastic leukemia: case report and review of the literature.Cancer Genet Cytogenet. 2006 Feb;165(1):75-8. Review.

Wells RJ, Arthur DC, Srivastava A, Heerema NA, Le Beau M, Alonzo TA, Buxton AB, Woods WG, Howells WB, Benjamin DR, Betcher DL, Buckley JD, Feig SA, Kim T, Odom LF, Ruymann FB, Smithson WA, Tannous R, Whitt JK, Wolff L, Tjoa T, Lampkin BC. Prognostic variables in newly diagnosed children and adolescents with acute myeloid leukemia: Children's Cancer Group Study 213.Leukemia. 2002 Apr;16(4):601-7.

Sande JE, Arceci RJ, Lampkin BC. Congenital and neonatal leukemia. Semin Perinatol. 1999 Aug;23(4):274-85. Review.

Mazewski C, Soukup S, Ballard E, Gotwals B, Lampkin B. Karyotype studies in 18 ependymomas with literature review of 107 cases. Cancer Genet Cytogenet. 1999 Aug;113(1):1-8. Review.

Arceci RJ, Reaman GH, Cohen AR, Lampkin BC. Position statement for the need to define pediatric hematology/oncology programs: a model of subspecialty care for chronic childhood diseases. Health Care Policy and Public Issues Committee of the American Society of Pediatric Hematology/Oncology.J Pediatr Hematol Oncol. 1998 Mar-Apr;20(2):98-103.

Klopfenstein K, Soukup S, Blough R, Mazewski C, Ballard E, Gotwals B, Lampkin B. Chromosome analyses in a rhabdoid tumor of the brain.Cancer Genet Cytogenet. 1997 Feb;93(2):152-6.

Miller CW, Aslo A, Won A, Tan M, Lampkin B, Koeffler HP. Alterations of the p53, Rb and MDM2 genes in osteosarcoma.J Cancer Res Clin Oncol. 1996;122(9):559-65.

George BA, Yanik G, Wells RJ, Martin LW, Soukup S, Ballard ET, Gartside PS, Lampkin BC. Growth patterns of human neuroblastoma xenografts and their relationship to treatment outcome.Cancer. 1993 Dec 1;72(11):3331-9.

Kadam P, Umerani A, Srivastava A, Masterson M, Lampkin B, Raza A. Combination of classical and interphase cytogenetics to investigate the biology of myeloid disorders: detection of masked monosomy 7 in AML.Leuk Res. 1993 Apr;17(4):365-74.

Pediatric leukemia and lymphoma; investigation of the role of small Rho GTPases in leukemogenesis and leukemic stem cell biology and their potential as therapeutic targets in acute myeloid leukemia; development of xenograft models for use in testing novel therapeutics

Mizukawa B, Wei J, Shrestha M, Wunderlich M, Chou FS, Griesinger A, Harris CE, Kumar AR, Zheng Y, Williams DA, Mulloy JC. Inhibition of Rac GTPase signaling and downstream pro-survival Bcl-2 proteins as combination targeted therapy in MLL-AF9 leukemia. Blood 118(19):5235-45, 2011.

Mizukawa B, George A, Pushkaran S, Weckbach L, Kalinyak K, Heubi JE, Kalfa TA.  Cooperating G6PD mutations associated with severe neonatal hyperbilirubinemia and cholestasis. Pediatr Blood Cancer 56(5): 840-2, 2011.

Wunderlich M, Chou FS, Link KA, Mizukawa B, Perry RL, Carroll M, Mulloy JC. AML xenograft efficiency is significantly improved in NOD/SCID-IL2RG mice constitutively expressing human SCF, GM-CSF, and IL-3.Leukemia, 2010; 24(10):1785-8.

Clinical Interests

Bone tumors; late effects of pediatric cancer therapy

Research Interests

Outcomes following cancer therapy and bone sarcomas

BA: Pre-Medical Sciences, Lehigh University, Bethlehem, PA, 1991.

MD: Medical College of Pennsylvania, Philadelphia, PA, 1995.

Internship and Residency: Pediatrics, Medical College of Virginia, Richmond, VA, 1998.

Fellowship Training: Hematology / Oncology / BMT, University of Minnesota, Minneapolis, MN, 2002.

MS: Clinical Research, University of Minnesota, Minneapolis, MN, 2002.

Certifications: Pediatrics, Pediatric Hematology / Oncology.

Nagarajan R, Kamruzzaman A, Ness KK, Marchese VG, Sklar C, Mertens A, Yasui Y, Robison LL, Marina N. Twenty years of follow-up of survivors of childhood osteosarcoma: a report from the childhood cancer survivor study. Cancer. 2011 Feb 1;117(3):625-34.

Rayburg M, Towbin A, Yin H, Maugans T, Maurer B, Nagarajan R, Weiss B. Langerhans cell histiocytosis in a patient with stage 4 neuroblastoma receiving oral fenretinide. Pediatr Blood Cancer. 2009 Dec;53(6):1111-3.

Nagarajan R. Quality of life (QOL) in patients with osteosarcoma. Recent Results Cancer Res. 2009;179:339-44. 

Nagarajan R, Mogil R, Neglia JP, Robison LL, Ness KK. Self-reported global function among adult survivors of childhood lower-extremity bone tumors: a report from the Childhood Cancer Survivor Study (CCSS). J Cancer Surviv. 2009 Mar;3(1):59-65.

Zebrack BJ, Zevon MA, Turk N, Nagarajan R, Whitton J, Robison LL, Zeltzer LK. Psychological distress in long-term survivors of solid tumors diagnosed in childhood: a report from the childhood cancer survivor study. Pediatr Blood Cancer. 2007 Jul;49(1):47-51. 

Khan K, Schwarzenberg SJ, Sharp H, Jessurun J, Gulbahce HE, Defor T, Nagarajan R. Diagnostic endoscopy in children after hematopoietic stem cell transplantation. Gastrointest Endosc. Sep 2006;64(3):379-85.

Schultz KA, Ness KK, Nagarajan R, Steiner ME. Adnexal masses in infancy and childhood. Clinical Obstetrics and Gynecology. Sep 2006; 49(3):464-79.

Nagarajan R, Clohisy D, Weigel B. New paradigms for therapy for osteosarcoma. Curr Oncol Rep. 2005 Nov;7(6):410-4.

Nagarajan R, Robison LL. Pregnancy outcomes in survivors of childhood cancer. J Natl Cancer Inst Monogr. 2005;(34):72-6.

Book Chapters

Spector LG, Ross JA, Nagarajan R. Epidemiology of bone and soft tissue sarcomas. In Pappo A (Ed.) Pediatric Bone and Soft Tissue Tumors. 1st ed. Berlin: Springer-Verlag, 2006.

Clinical Interests

Leukemia; lymphoma

Research Interests

Molecular etiology of pediatric leukemias, especially in children with Down syndrome; novel therapeutics for relapsed and high-risk leukemia and lymphoma

Spicakova T, O'Brien MM, Duran GE, Sweet-Cordero A, Sikic BI. Expression and silencing of the microtubule-associated protein Tau in breast cancer cells. Mol Cancer Ther. 2010 Nov;9(11):2970-81.

O'Brien MM, Lacayo NJ, Lum BL, Kshirsagar S, Buck S, Ravindranath Y, Bernstein M, Weinstein H, Chang MN, Arceci RJ, Sikic BI, Dahl GV. Phase I study of valspodar (PSC-833) with mitoxantrone and etoposide in refractory and relapsed pediatric acute leukemia: a report from the Children's Oncology Group. Pediatr Blood Cancer. 2010 May;54(5):694-702.

O'Brien MM, Donaldson SS, Balise RR, Whittemore AS, Link MP. Second malignant neoplasms in survivors of pediatric Hodgkin's lymphoma treated with low-dose radiation and chemotherapy. J Clin Oncol. 2010 Mar 1;28(7):1232-9.   

Jeng MR, O’Brien M, Wong W, Zoland J, Lea J, Tang N, Glader B.  Monthly recombinant tissue plasminogen activator administration to implantable central venous access devices decreases infections in children with haemophilia. Haemophilia. 2009 Nov 15(6):1272-80.

Donaldson SS, O'Brien MM. Understanding the risk of second malignant tumors in children with Hodgkin's disease. Int J Radiat Oncol Biol Phys. 2008 Sep 1;72(1):4-5.

O’Brien MM, Lee-Kim Y, George T, McClain K, Twist C, Jeng M.   Precursor B-cell acute lymphoblastic leukemia presenting with hemophagocytic lymphohistiocytosis: case series and review of the literature.  Pediatr Blood Cancer. 2008 Feb 50(2):381-3

O’Brien MM, Taub JW, Chang MN, Massey GV, Stine KC, Raimondi SC, Becton D, Ravindranath Y, Dahl GV.  Cardiomyopathy in children with Down syndrome treated for acute myeloid leukemia: a report from the Children’s Oncology Group Study POG 9421.  J Clin Oncol. 2008 Jan 26(3):414-420.

Clinical Interests

Acute myeloid leukemia; neuroblastoma; PNET / Ewing's sarcoma and osteosarcoma; new anticancer drug development; Phase I clinical trials

Research Interests

Molecular etiology and pharmacogenetics of pediatric cancers; Down syndrome-associated leukemia; new anticancer drug development

MD: University of Michigan, Ann Arbor, MI, 1980.

Residency: University of Minnesota Medical School, Minneapolis, MN, 1983.

Fellowship: University of Minnesota Medical School, Minneapolis, MN, 1986.

Postdoctoral: University of Minnesota Medical School, Minneapolis, MN, 1986.

Certification: Pediatrics, 1989; Hematology/Oncology, 1990.

Dorris K, Fouladi M, Davies SM, Perentesis JP, Lawrence JM, Chow LM, Assa'ad A, Uygungil B, Jodele S. . Severe Allergic Reactions to Thiol-based Cytoprotective Agents Mesna and Amifostine in a Child With a Supratentorial Primitive Neuroectodermal Tumor.J Pediatr Hematol Oncol. 2011 Jun 3.

Davies SM, Perentesis JP. Tribute: the American Society of Pediatric Hematology/Oncology (ASPHO), 2011 Distinguished Career Award goes to Dr. William G. Woods. Pediatr Blood Cancer. 2011 Jun;56(6):895-6.

Phillips CL, Gerbing R, Alonzo T, Perentesis JP, Harley IT, Meshinchi S, Bhatla D, Radloff G, Davies SM. MDM2 polymorphism increases susceptibility to childhood acute myeloid leukemia: a report from the Children's Oncology Group.Pediatr Blood Cancer. 2010 Aug;55(2):248-53.

Wagner LM, Perentesis JP, Reid JM, Ames MM, Safgren SL, Nelson MD Jr, Ingle AM, Blaney SM, Adamson PC. Phase I trial of two schedules of vincristine, oral irinotecan, and temozolomide (VOIT) for children with relapsed or refractory solid tumors: a Children's Oncology Group phase I consortium study.Pediatr Blood Cancer. 2010 Apr;54(4):538-45.

Mehta PA, Gerbing RB, Alonzo TA, Elliott JS, Zamzow TA, Combs M, Stover E, Ross JA, Perentesis JP, Meschinchi S, Lange BJ, Davies SM. FAS promoter polymorphism: outcome of childhood acute myeloid leukemia. A children's oncology group report.Clin Cancer Res. 2008 Dec 1;14(23):7896-9.

Bhatla D, Gerbing RB, Alonzo TA, Conner H, Ross JA, Meshinchi S, Zhai X, Zamzow T, Mehta PA, Geiger H, Perentesis J, Davies SM. Cytidine deaminase genotype and toxicity of cytosine arabinoside therapy in children with acute myeloid leukemia.Br J Haematol. 2009 Feb;144(3):388-94.

Geller JI, Wall D, Perentesis J, Blaney SM, Bernstein M; Pediatric Oncology Group study 9376. Phase I study of paclitaxel with standard dose ifosfamide in children with refractory solid tumors: a Pediatric Oncology Group study (POG 9376).Pediatr Blood Cancer. 2009 Mar;52(3):346-50.

Johansson G, Mahller YY, Collins MH, Kim MO, Nobukuni T, Perentesis J, Cripe TP, Lane HA, Kozma SC, Thomas G, Ratner N. Effective in vivo targeting of the mammalian target of rapamycin pathway in malignant peripheral nerve sheath tumors.Mol Cancer Ther. 2008 May;7(5):1237-45.

Bhatla D, Gerbing RB, Alonzo TA, Mehta PA, Deal K, Elliott J, Meshinchi S, Geiger H, Perentesis JP, Lange BJ, Davies SM; Children's Oncology Group. DNA repair polymorphisms and outcome of chemotherapy for acute myelogenous leukemia: a report from the Children's Oncology Group.Leukemia. 2008 Feb;22(2):265-72.

Mo J, Lampkin B, Perentesis J, Poole L, Bao L. Translocation (8;18;16)(p11;q21;p13). A new variant of t(8;16)(p11;p13) in acute monoblastic leukemia: case report and review of the literature. Cancer Genet Cytogenet. 2006 Feb;165(1):75-8. Review.

Leukemia and lymphoma; leukemia pharmacogenetics; translational and clinical development of new drugs for high-risk and relapsed leukemias

MD: Indiana University School of Medicine, Indianapolis, IN, 2002.

Residency: Pediatrics, Children’s Memorial Hospital, Chicago, IL, 2005.

Fellowship: Pediatric Hematology/Oncology, Cincinnati Children’s Hospital Medical Center, 2010.

Certification: Pediatrics, 2005; Pediatric Hematology/Oncology, 2011.

BA: University of Cincinnati, Cincinnati, OH, 2004.

BSN: University of Cincinnati, Cincinnati, OH, 2007.

MSN: University of Cincinnati, Cincinnati, OH, 2011. 

CNP Certification: Acute Care Pediatric Nurse Practitioner, 2011.

Neuroblastoma; gene therapy; sarcoma; neuro-oncology

MD: University of Kentucky, Lexington, KY 1991.

Residency: Pediatrics, University of Tennessee, Memphis, TN.

Fellowship: Pediatric Hematology/Oncology, St. Jude Children's Research Hospital, Memphis, TN.

Certification: Pediatrics, 1994.

Certification: Pediatric Hematology/Oncology, 2004.

Wagner LM, Gelfand MJ, Laor T, Ryckman FC, Al-Ghawi H, Bove KE. A Welcome Surprise: Nodular Fasciitis Presenting as Soft Tissue Sarcoma. J Pediatr Hematol Oncol. 2010 Oct 21.

Wagner LM. Oral irinotecan for treatment of pediatric solid tumors: ready for prime time?Pediatr Blood Cancer. 2010 May;54(5):661-2.

Wagner LM, Perentesis JP, Reid JM, Ames MM, Safgren SL, Nelson MD Jr, Ingle AM, Blaney SM, Adamson PC. Phase I trial of two schedules of vincristine, oral irinotecan, and temozolomide (VOIT) for children with relapsed or refractory solid tumors: a Children's Oncology Group phase I consortium study. Pediatr Blood Cancer. 2010 Apr;54(4):538-45.

Wagner LM, Danks MK. New therapeutic targets for the treatment of high-risk neuroblastoma. J Cell Biochem. 2009 May 1;107(1):46-57.

Wagner LM, Villablanca JG, Stewart CF, Crews KR, Groshen S, Reynolds CP, Park JR, Maris JM, Hawkins RA, Daldrup-Link HE, Jackson HA, Matthay KK. Phase I trial of oral irinotecan and temozolomide for children with relapsed high-risk neuroblastoma: a new approach to neuroblastoma therapy consortium study. J Clin Oncol. 2009 Mar 10;27(8):1290-6.

Wagner LM, Crews KR, Stewart CF, Rodriguez-Galindo C, McNall-Knapp RY, Albritton K, Pappo AS, Furman WL. Reducing irinotecan-associated diarrhea in children. Pediatr Blood Cancer. 2008 Feb;50(2):201-7.

Wagner LM, Garrett JK, Ballard ET, Hill DA, Perry A, Biegel JA, Collins MH. Malignant rhabdoid tumor mimicking hepatoblastoma: a case report and literature review. Pediatr Dev Pathol. 2007 Sep-Oct;10(5):409-15.

Wagner LM, McLendon RE, Yoon KJ, Weiss BD, Billups CA, Danks MK. Targeting methylguanine-DNA methyltransferase in the treatment of neuroblastoma. Clin Cancer Res. 2007 Sep 15;13(18 Pt 1):5418-25.

Will E, Bailey J, Schuesler T, Modlich U, Balcik B, Burzynski B, Witte D, Layh-Schmitt G, Rudolph C, Schlegelberger B, von Kalle C, Baum C, Sorrentino BP, Wagner LM, Kelly P, Reeves L, Williams DA. Importance of murine study design for testing toxicity of retroviral vectors in support of phase I trials. Mol Ther. 2007 Apr;15(4):782-91.
 

MD: Northwestern University Medical School, Chicago, IL, 1993.

Residency and Chief Residency: Pediatrics, University of California, San Francisco, CA, 1993-1997.

Fellowship: Pediatric Hematology/Oncology, University of California, San Francisco, CA, 1997-2000.

Certification: National Medical Board; Pediatrics;1996, 2002; Pediatric Hematology-Onoclogy, 2000, 2007

Hummel T, Anyane-Yeboa A, Mo J, Towbin A, Weiss B. Response of NF1-related plexiform neurofibroma to High-Dose Carboplatin. Pediatr Blood Cancer. 2011 Mar;56(3):488-90.

Rayburg M, Towbin A, Yin H, Maugans T, Maurer B, Nagarajan R, Weiss B. Langerhans cell histiocytosis in a patient with stage 4 neuroblastoma receiving oral fenretinide. Pediatr Blood Cancer. 2009 Dec;53(6):1111-3. 

Weiss B, Geiger H, Davies SM. Possible leukemogenic potential of temozolomide. Pediatr Blood Cancer. 2009 Apr;52(4):553. 

Chan RJ, Cooper T, Kratz CP, Weiss B, Loh ML. Juvenile myelomonocytic leukemia: a report from the 2nd International JMML Symposium. Leuk Res. 2009 Mar;33(3):355-62.

Wagner LM, McLendon RE, Yoon KJ, Weiss BD, Billups CA, Danks MK. Targeting methylguanine-DNA methyltransferase in the treatment of neuroblastoma. Clin Cancer Res. 2007 Sep 15;13(18 Pt 1):5418-25. 

Geiger H, Schleimer D, Nattamai KJ, Dannenmann SR, Davies SM, Weiss BD. Mutagenic potential of temozolomide in bone marrow cells in vivo. Blood. 2006 Apr 1;107(7):3010-1.

Book Chapters

Weiss B and Shannon K. Preliminary Examples of Preclinical Trials. In Mouse Models of Cancer, Eric Holland (Ed). Wiley-Liss, 2004.

Squamous cell carcinoma; mechanisms by which the HPV oncogenes subvert the host cell machinery to promote abnormal cell growth and cancer; role of specific cellular HPV targets in viral replication and cellular transformation

Visit the Wells Lab.

Spence JR, Mayhew CN, Rankin SA, Kuhar MF, Vallance JE, Tolle K, Hoskins EE, Kalinichenko VV, Wells SI, Zorn AM, Shroyer NF, Wells JM. Directed differentiation of human pluripotent stem cells into intestinal tissue in vitro. Nature. 2010 Dec 12.

Meyer SE, Peace BE, Bahassi el M, Kavanaugh GM, Wagh PK, Robbins SB, Yin M, Wells SI, Zinser GM, Stambrook PJ, Waltz SE. Chk2*1100delC Acts in synergy with the Ron receptor tyrosine kinase to accelerate mammary tumorigenesis in mice. Cancer Lett. 2010 Oct 28;296(2):186-93.

Blanchard C, Stucke EM, Burwinkel K, Caldwell JM, Collins MH, Ahrens A, Buckmeier BK, Jameson SC, Greenberg A, Kaul A, Franciosi JP, Kushner JP, Martin LJ, Putnam PE, Abonia JP, Wells SI, Rothenberg ME. Coordinate interaction between IL-13 and epithelial differentiation cluster genes in eosinophilic esophagitis. J Immunol. 2010 Apr 1;184(7):4033-41. Epub 2010 Mar 5. 

Wise-Draper TM, Mintz-Cole RA, Morris TA, Simpson DS, Wikenheiser-Brokamp KA, Currier MA, Cripe TP, Grosveld GC, Wells SI. Overexpression of the cellular DEK protein promotes epithelial transformation in vitro and in vivo. Cancer Res. 2009 Mar 1;69(5):1792-9. 

Hoskins EE, Morris TA, Higginbotham JM, Spardy N, Cha E, Kelly P, Williams DA, Wikenheiser-Brokamp KA, Duensing S, Wells SI. Fanconi anemia deficiency stimulates HPV-associated hyperplastic growth in organotypic epithelial raft culture. Oncogene. 2009 Feb 5;28(5):674-85.

Wise-Draper TM, Morreale RJ, Morris TA, Mintz-Cole RA, Hoskins EE, Balsitis SJ, Husseinzadeh N, Witte DP, Wikenheiser-Brokamp KA, Lambert PF, Wells SI. DEK proto-oncogene expression interferes with the normal epithelial differentiation program. Am J Pathol. 2009 Jan;174(1):71-81. 

Bourgo RJ, Braden WA, Wells SI, Knudsen ES. Activation of the retinoblastoma tumor suppressor mediates cell cycle inhibition and cell death in specific cervical cancer cell lines. Mol Carcinog. 2009 Jan;48(1):45-55.

Hoskins EE, Gunawardena RW, Habash KB, Wise-Draper TM, Jansen M, Knudsen ES, Wells SI. Coordinate regulation of Fanconi anemia gene expression occurs through the Rb/E2F pathway. Oncogene. 2008 Aug 14;27(35):4798-808. 

Wise-Draper TM, Wells SI. Papillomavirus E6 and E7 proteins and their cellular targets. Front Biosci. 2008 Jan 1;13:1003-17. Review.

Blanchard C, Mingler MK, Vicario M, Abonia JP, Wu YY, Lu TX, Collins MH, Putnam PE, Wells SI, Rothenberg ME. IL-13 involvement in eosinophilic esophagitis: transcriptome analysis and reversibility with glucocorticoids. J Allergy Clin Immunol. 2007 Dec;120(6):1292-300.

Clinical Interests

Fanconi Anemia; cell cycle check points; genetic instability; replication stress; relationship of DNA repair and chromatin; mitosis; cell biology

Visit the Andreassen Lab.

BS: Willamette University, Salem, Oregon, 1984

PhD: University of Washington, Seattle, Washington, 1995

Clinical Interests

Leukemia

Research Interests

Structure and function analysis of tyrosine kinases involved in the pathogenesis of leukemia; molecular basis of “oncogene addiction”;  modeling of human leukemia in mice using ES and patient derived iPS Cells

Azam M, Powers JT, Einhorn W, Huang WS, Shakespeare WC, Zhu X, Dalgarno D, Clackson T, Sawyer TK, Daley GQ. AP24163 inhibits the gatekeeper mutant of BCR-ABL and suppresses in vitro resistance. Chem Biol Drug Des. 2010 Feb;75(2):223-7.  

Zhang J, Adrián FJ, Jahnke W, Cowan-Jacob SW, Li AG, Iacob RE, Sim T, Powers J, Dierks C, Sun F, Guo GR, Ding Q, Okram B, Choi Y, Wojciechowski A, Deng X, Liu G, Fendrich G, Strauss A, Vajpai N, Grzesiek S, Tuntland T, Liu Y, Bursulaya B, Azam M, Manley PW, Engen JR, Daley GQ, Warmuth M, Gray NS. Targeting Bcr-Abl by combining allosteric with ATP-binding-site inhibitors. Nature. 2010 Jan 28;463(7280):501-6.

Nardi V, Azam M, Neverias O, Daley GQ. Immune-Mediated Protection AgainstBCR/ABL-Induced Leukemia: A Common Pathway Shared between IRF8/ICSBP and IFN alpha and beta. Blood. 2009.

Viswanathan SR, Powers JT, Einhorn W, Hoshida Y, Ng TL, Toffanin S, O'Sullivan M, Lu J, Phillips LA, Lockhart VL, Shah SP, Tanwar PS, Mermel CH, Beroukhim R, Azam M, Teixeira J, Meyerson M, Hughes TP, Llovet JM, Radich J, Mullighan CG, Golub TR, Sorensen PH, Daley GQ. Lin28 promotes transformation and is associated with advanced human malignancies. Nature Genetics. 2009 Jul;41(7):843-8.

Azam M, Seeliger M, Gray S, Kuriyan J, Daley GQ. Activation of tyrosine kinases by the mutation of gatekeeper residue. Nat Struct Mol Biol. 2008 Oct;15(10):1109-18.

Raz T, Nardi V, Azam M, Cortes J, Daley GQ. Farnesyl transferase inhibitor resistance probed by target mutagenesis. Blood. 2007 Sep 15;110(6):2102-9.

Azam M, Nardi V, Shakespeare WC, Metcalf CA 3rd, Bohacek RS, Wang Y, Sundaramoorthi R, Sliz P, Veach DR, Bornmann WG, Clarkson B, Dalgarno DC, Sawyer TK, Daley GQ. Activity of dual SRC-ABL inhibitors highlights role of BCR/ABL kinase dynamics in drug resistance. Proc Natl Acad Sci U S A. 2006 Jun 13;103(24):9244-9.  

Azam M, and Daley GQ. Anticipating Clinical Resistance to Target-directed Agents: BCR-ABL an example. Molecular Diagnosis and Therapy. 2006;10(2):67-76.

Clinical Interests

Retrovirus biology and vectorology; stem cell biology; gene therapy; leukemia biology

Arumugam PI, Higashimoto T, Urbinati F, Modlich U, Nestheide S, Xia P, Fox C, Corsinotti A, Baum C, Malik P. Genotoxic potential of lineage-specific lentivirus vectors carrying the beta-globin locus control region. Mol Ther. 2009 Nov;17(11):1929-37.

Milsom MD, Jerabek-Willemsen M, Harris CE, Schambach A, Broun E, Bailey J, Jansen M, Schleimer D, Nattamai K, Wilhelm J, Watson A, Geiger H, Margison GP, Moritz T, Baum C, Thomale J, Williams DA.  Reciprocal relationship between O6-methylguanine-DNA methyltransferase P140K expression level and chemoprotection of hematopoietic stem cells. Cancer Res. 2008 Aug 1;68(15):6171-80.

Will E, Bailey J, Schuesler T, Modlich U, Balcik B, Burzynski B, Witte D, Layh-Schmitt G, Rudolph C, Schlegelberger B, von Kalle C, Baum C, Sorrentino BP, Wagner LM, Kelly P, Reeves L, Williams DA. Importance of murine study design for testing toxicity of retroviral vectors in support of phase I trials. Mol Ther. 2007 Apr;15(4):782-91. 

Will E, Speidel D, Wang Z, Ghiaur G, Rimek A, Schiedlmeier B, Williams DA, Baum C, Ostertag W, Klump H. HOXB4 inhibits cell growth in a dose-dependent manner and sensitizes cells towards extrinsic cues. Cell Cycle. 2006 Jan;5(1):14-22. 

Schambach, A, Schiedlmeier B, Kuhicke K, Verstegen M, Margison G, Li Z, Kamino K, Bahne J, Alexandrov A, Hermann FG, von Laer D, Baum C. Towards hematopoietic stem cell-mediated protection against infection with human immunodeficiency virus.Gene Therapy 2006; 13:1037-1047.

Von Kalle C, Fehse B, Layh-Schmitt G, Schmidt M, Kelly P, Baum C. Stem cell clonality and genotoxicity in hematopoietic cells: gene activation side effects should be avoidable. Semin Hematol. 2004 Oct;41(4):303-18. 

Schambach A, Galla M, Modlich U, Will E, Chandra S, Reeves L, Colbert M, Williams DA, von Kalle C, Baum C. Lentiviral vectors pseudotyped with murine ecotropic evelope: Increased biosafety and convenience for preclinical research. Experimental Hematology. 2006; 34-588-592.

Schambach A, Bohne J, Baum C, Hermann FG, Egerer L, von Laer D, Giroglou T. Woodchuck hepatitis virus posttranscriptional regulatory element deleted from X protein enhances retroviral vector titer and expression. Gene Therapy. 2006; 13:241-246.

Kustikova O, Fehse B, Modlich U, Düllmann J, Kamino K, von Neuhoff N, Schlegelberger B, Li Z, Baum C. Clonal dominance of hematopoietic stem cells triggered by retroviral gene marking. Science. 2005 May 20;308(5725):1171-4.

Galla M, Will E, Kraunus J, Chen L, Baum C. Retroviral pseudotransduction for targeted cell manipulation. Mol Cell. 2004 Oct 22;16(2):309-15. 

Hematopoietic stem cell proliferation and differentiation

Visit the Cancelas Lab.
 

MD: Autonomous University of Madrid, Spain, 1989.

Residency: Hematology and Hematotherapy, University of Alcala de Henares, Madrid, Spain, 1993.

PhD: Faculty of Medicine, University of Alcala de Henares, Madrid, Spain, 1996.

Molecular genetics of plasminogen activation in development, hemostasis, and tumor progression; molecular genetics and biological role of plasminogen activation in development, hemostasis, wound repair, and disease

Mulloy JC, Cancelas JA, Filippi MD, Kalfa TA, Guo F, Zheng Y. Rho GTPases in hematopoiesis and hemopathies. Blood. 2010 Feb 4;115(5):936-47.

Szczur K, Zheng Y, Filippi MD. The small Rho GTPase Cdc42 regulates neutrophil polarity via CD11b integrin signaling. Blood. 2009 Nov 12;114(20):4527-37.

Xu H, Eleswarapu S, Geiger H, Szczur K, Daria D, Zheng Y, Settleman J, Srour EF, Williams DA, Filippi MD. Loss of the Rho GTPase activating protein p190-B enhances hematopoietic stem cell engraftment potential. Blood. 2009 Oct 22;114(17):3557-66.

Gu Y, Harley IT, Henderson LB, Aronow BJ, Vietor I, Huber LA, Harley JB, Kilpatrick JR, Langefeld CD, Williams AH, Jegga AG, Chen J, Wills-Karp M, Arshad SH, Ewart SL, Thio CL, Flick LM, Filippi MD, Grimes HL, Drumm ML, Cutting GR, Knowles MR, Karp CL. Identification of IFRD1 as a modifier gene for cystic fibrosis lung disease. Nature. 2009 Apr 23;458(7241):1039-42.

Monk KR, Wu J, Williams JP, Finney BA, Fitzgerald ME, Filippi MD, Ratner N. Mast cells can contribute to axon-glial dissociation and fibrosis in peripheral nerve. Neuron Glia Biol. 2007 Aug;3(3):233-44.

Daria D, Filippi MD, Knudsen ES, Faccio R, Li Z, Kalfa T, Geiger H. The retinoblastoma tumor suppressor is a critical intrinsic regulator for hematopoietic stem and progenitor cells under stress. Blood. 2008 Feb 15;111(4):1894-902.

Uchida K, Beck DC, Yamamoto T, Berclaz PY, Abe S, Staudt MK, Carey BC, Filippi MD, Wert SE, Denson LA, Puchalski JT, Hauck DM, Trapnell BC. GM-CSF autoantibodies and neutrophil dysfunction in pulmonary alveolar proteinosis.N Engl J Med. 2007 Feb 8;356(6):567-79.

Filippi MD, Szczur K, Harris CE, Berclaz PY. Rho GTPase Rac1 is critical for neutrophil migration into the lung. Blood. 2007 Feb 1;109(3):1257-64.

Szczur K, Xu H, Atkinson S, Zheng Y, Filippi MD. Rho GTPase CDC42 regulates directionality and random movement via distinct MAPK pathways in neutrophils. Blood. 2006 Dec 15;108(13):4205-13.

Wang L, Yang L, Filippi MD, Williams DA, Zheng Y. Genetic deletion of Cdc42GAP reveals a role of Cdc42 in erythropoiesis and hematopoietic stem/progenitor cell survival, adhesion, and engraftment. Blood. 2006 Jan 1;107(1):98-105.

Horowitz NA, Blevins EA, Miller WM, Perry AR, Talmage KE, Mullins ES, Flick MJ, Queiroz KC, Shi K, Spek CA, Conway EM, Monia BP, Weiler H, Degen JL, Palumbo JS. Thrombomodulin is a determinant of metastasis through a mechanism linked to the thrombin binding domain but not the lectin-like domain. Blood. 2011 Jul 25.

Raghu H, Flick MJ. Targeting the Coagulation Factor Fibrinogen for Arthritis Therapy. Curr Pharm Biotechnol. 2011 Mar 14.

Flick MJ, Chauhan AK, Frederick M, Talmage KE, Kombrinck KW, Miller W, Mullins ES, Palumbo JS, Zheng X, Esmon NL, Esmon CT, Thornton S, Becker A, Pelc LA, Di Cera E, Wagner DD, Degen JL. The development of inflammatory joint disease is attenuated in mice expressing the anticoagulant prothrombin mutant W215A/E217A. Blood. 2011 Jun 9;117(23):6326-37.

Steinbrecher KA, Horowitz NA, Blevins EA, Barney KA, Shaw MA, Harmel-Laws E, Finkelman FD, Flick MJ, Pinkerton MD, Talmage KE, Kombrinck KW, Witte DP, Palumbo JS. Colitis-associated cancer is dependent on the interplay between the hemostatic and inflammatory systems and supported by integrin alpha(M)beta(2) engagement of fibrinogen. Cancer Res. 2010 Apr 1;70(7):2634-43.

Lykens JE, Terrell CE, Zoller EE, Divanovic S, Trompette A, Karp CL, Aliberti J, Flick MJ, Jordan MB. Mice with a selective impairment of IFN-gamma signaling in macrophage lineage cells demonstrate the critical role of IFN-gamma-activated macrophages for the control of protozoan parasitic infections in vivo. J Immunol. 2010 Jan 15;184(2):877-85.

Mullins ES, Kombrinck KW, Talmage KE, Shaw MA, Witte DP, Ullman JM, Degen SJ, Sun W, Flick MJ, Degen JL. Genetic elimination of prothrombin in adult mice is not compatible with survival and results in spontaneous hemorrhagic events in both heart and brain. Blood. 2009 Jan 15;113(3):696-704.

Palumbo JS, Barney KA, Blevins EA, Shaw MA, Mishra A, Flick MJ, Kombrinck KW, Talmage KE, Souri M, Ichinose A, Degen JL. Factor XIII transglutaminase supports hematogenous tumor cell metastasis through a mechanism dependent on natural killer cell function. J Thromb Haemost. 2008 May;6(5):812-9.

Flick MJ, LaJeunesse CM, Talmage KE, Witte DP, Palumbo JS, Pinkerton MD, Thornton S, Degen JL. Fibrin(ogen) exacerbates inflammatory joint disease through a mechanism linked to the integrin alphaMbeta2 binding motif. J Clin Invest. 2007 Nov ;117(11):3224-35.

Palumbo JS, Talmage KE, Massari JV, La Jeunesse CM, Flick MJ, Kombrinck KW, Hu Z, Barney KA, Degen JL. Tumor cell-associated tissue factor and circulating hemostatic factors cooperate to increase metastatic potential through natural killer cell-dependent and-independent mechanisms. Blood. 2007 Jul 1;110(1):133-41.

Palumbo JS, Talmage KE, Massari JV, La Jeunesse CM, Flick MJ, Kombrinck KW, Jirousková M, Degen JL. Platelets and fibrin(ogen) increase metastatic potential by impeding natural killer cell-mediated elimination of tumor cells. Blood. 2005 Jan 1;105(1):178-85.

Hematopoietic stem cells; genetics; aging/longevity; plasticity of stem cells; mobilization; DNA-repair

Visit the Geiger Lab.
 

Ryan MA, Nattamai KJ, Xing E, Schleimer D, Daria D, Sengupta A, Köhler A, Liu W, Gunzer M, Jansen M, Ratner N, Le Cras TD, Waterstrat A, Van Zant G, Cancelas JA, Zheng Y, Geiger H. Pharmacological inhibition of EGFR signaling enhances G-CSF-induced hematopoietic stem cell mobilization. Nat Med. 2010 Oct;16(10):1141-6.

Kalfa TA, Pushkaran S, Zhang X, Johnson JF, Pan D, Daria D, Geiger H, Cancelas JA, Williams DA, Zheng Y. Rac1 and Rac2 GTPases are necessary for early erythropoietic expansion in the bone marrow but not in the spleen. Haematologica. 2010 Jan;95(1):27-35.

Xu H, Eleswarapu S, Geiger H, Szczur K, Daria D, Zheng Y, Settleman J, Srour EF, Williams DA, Filippi MD. Loss of the Rho GTPase activating protein p190-B enhances hematopoietic stem cell engraftment potential. Blood. 2009 Oct 22;114(17):3557-66.

Geiger H, Rudolph KL. Aging in the lympho-hematopoietic stem cell compartment. Trends Immunol. 2009 Jul;30(7):360-5. Epub 2009 Jun 18. Review.

Geiger H, David S, Nattamai KJ, Jan V. Quantification of genomic mutations in murine hematopoietic cells. Methods Mol Biol. 2009;506:423-36.

Williams JP, Wu J, Johansson G, Rizvi TA, Miller SC, Geiger H, Malik P, Li W, Mukouyama YS, Cancelas JA, Ratner N. Nf1 mutation expands an EGFR-dependent peripheral nerve progenitor that confers neurofibroma tumorigenic potential. Cell Stem Cell. 2008 Dec 4;3(6):658-69.

Bhatla D, Gerbing RB, Alonzo TA, Conner H, Ross JA, Meshinchi S, Zhai X, Zamzow T, Mehta PA, Geiger H, Perentesis J, Davies SM. Cytidine deaminase genotype and toxicity of cytosine arabinoside therapy in children with acute myeloid leukemia. Br J Haematol. 2009 Feb;144(3):388-94.

Milsom MD, Jerabek-Willemsen M, Harris CE, Schambach A, Broun E, Bailey J, Jansen M, Schleimer D, Nattamai K, Wilhelm J, Watson A, Geiger H, Margison GP, Moritz T, Baum C, Thomale J, Williams DA. Reciprocal relationship between O6-methylguanine-DNA methyltransferase P140K expression level and chemoprotection of hematopoietic stem cells. Cancer Res. 2008 Aug 1;68(15):6171-80.

Diwan A, Koesters AG, Capella D, Geiger H, Kalfa TA, Dorn GW 2nd. Targeting erythroblast-specific apoptosis in experimental anemia. Apoptosis. 2008 Aug;13(8):1022-30.

Daria D, Filippi MD, Knudsen ES, Faccio R, Li Z, Kalfa T, Geiger H. The retinoblastoma tumor suppressor is a critical intrinsic regulator for hematopoietic stem and progenitor cells under stress. Blood. 2008 Feb 15;111(4):1894-902.

Clinical Interests

Diagnostic assay for the identification of complementation groups on specimens from patients diagnosed with Fanconi Anemia

Research Interests

Phase I gene transfer / cell therapy trials; investigational new drug (IND) submissions to the FDA; evaluate new vectors with respect to safety and efficacy; develop tailored assays for trial monitoring; stat Endotoxin testing service to support cell therapy trials

Singh TR, Bakker ST, Agarwal S, Jansen M, Grassman E, Godthelp BC, Ali AM, Du CH, Rooimans MA, Fan Q, Wahengbam K, Steltenpool J, Andreassen PR, Williams DA, Joenje H, de Winter JP, Meetei AR. Impaired FANCD2 monoubiquitination and hypersensitivity to camptothecin uniquely characterize Fanconi anemia complementation group M.  Blood. 2009 Jul 2;114(1):174-80. Epub 2009 May 7.

Balcik B, Grassman E, Reeves L. Database setup for preclinical studies of gene-modified hematopoiesis.Methods Mol Biol. 2009;506:467-76.

Schuesler T, Reeves L, Kalle C, Grassman E. Copy number determination of genetically-modified hematopoietic stem cells. Methods Mol Biol. 2009;506:281-98.

Will E, Bailey J, Schuesler T, Modlich U, Balcik B, Burzynski B, Witte D, Layh-Schmitt G, Rudolph C, Schlegelberger B, Von Kalle C, Baum C, Sorrentino BP, Wagner LM, Kelly P, Reeves L, Williams DA.Importance of murine study design for testing toxicity of retroviral vectors in support of Phase I trials. Mol. Ther. 2007 Apr 15(4):782-91.

Li Z, Kustikova OS, Kamino K, Neumann T, Rhein M, Grassman E, Fehse B, Baum C. Mutagenesis by Replication-Deficient Retroviral Vectors Encoding the Large T Oncogene. Annals of the New York Academy of Sciences. 2007 1106:95-113.

Thornhill SI, Schambach A, Howe SJ, Ulaganathan M, Grassman E, Williams D, Schiedlmeier B, Sebire NJ, Gaspar HB, Kinnon C, Baum C, Thrasher AJ. Self-inactivating gammaretroviral vectors for gene therapy of X-linked severe combined immunodeficiency. Mol Ther. 2008 16(3):590-8.

Zychlinski D, Schambach A, Modlich U, Maetzig T, Meyer J, Grassman E, Mishra A, Baum C. Physiological promoters reduce the genotoxic risk of integrating gene vectors. Mol Ther. 2008 16(4):718-25.

Rho GTPases and T and B lymphocyte development and function

Guo F, Cancelas JA, Hildeman D, Williams DA, Zheng Y. Rac GTPase isoforms, Rac1 and Rac2, play a redundant and crucial role in T-cell development. Blood, 2008, 112 (5):1767.

Guo F, Zhang S, Tripathi P, Mattner J, Phelan J, Sproles A, Mo J, Wills-Karp M, Grimes HL, Hildeman D, Zheng Y. Distinct roles of Cdc42 in thymopoiesis and effector and memory T cell differentiation. PLoS One, 2011, 6(3):e18002.

Shang X, Cancelas JA, Li L, Guo F, Liu W, Johnson JF, Ficker A, Daria D, Geiger H, Ratner N, Zheng Y. R-Ras and Rac GTPase crosstalk regulates hematopoietic progenitor cell migration, homing and mobilization. Journal of Biological Chemistry, 2011, 286(27):24068.

Sipes NS, Feng Y, Guo F, Lee HO, Chou FS, Cheng J, Mulloy J, Zheng Y. Cdc42 regulates extracellular matrix remodeling in three dimensions. Journal of Biological Chemistry, 2011, 286(42):36469-77. 

Guo F, Hildeman D, Tripathi P, Velu CS, Grimes HL, Zheng Y. Coordination of IL-7 receptor and T-cell receptor signaling by cell-division cycle 42 in T-cell homeostasis. Proc Natl Acad Sci U S A. 2010 Oct 26;107(43):18505-10.

Bosco EE, Ni W, Wang L, Guo F, Johnson JF, Zheng Y. Rac1 targeting suppresses p53 deficiency-mediated lymphomagenesis. Blood. 2010 Apr 22;115(16):3320-8.

Mulloy JC, Cancelas JA, Filippi MD, Kalfa TA, Guo F, Zheng Y. Rho GTPases in hematopoiesis and hemopathies. Blood. 2010 Feb 4;115(5):936-47.

Guo F, Velu CS, Grimes HL, Zheng Y. Rho GTPase Cdc42 is essential for B-lymphocyte development and activation. Blood. 2009 Oct 1;114(14):2909-16

Zhang X, Shang X, Guo F, Murphy K, Kirby M, Kelly P, Reeves L, Smith FO, Williams DA, Zheng Y, Pang Q. Defective homing is associated with altered Cdc42 activity in cells from patients with Fanconi anemia group A. Blood. 2008 Sep 1;112(5):1683-6.

Guo F, Debidda M, Yang L, Williams DA, Zheng Y. Genetic deletion of Rac1 GTPase reveals its critical role in actin stress fiber formation and focal adhesion complex assembly. J Biol Chem. 2006 Jul 7;281(27):18652-9. 

Komurov K, Ram PT. Patterns of human gene expression variance show strong associations with signaling network hierarchy. BMC Syst Biol. 2010 Nov 12;4:154.

Komurov K, White MA, Ram PT. Use of data-biased random walks on graphs for the retrieval of context-specific networks from genomic data. PLoS Comput Biol. 2010 Aug 19;6(8).

Taube JH*, Herschkowitz JI*, Komurov K*, Zhou AY, Gupta S, Yang J, Hartwell K, Onder TT, Gupta PB, Evans KW, Hollier BG, Ram PT, Lander ES, Rosen JM, Weinberg RA, Mani SA. Core epithelial-to-mesenchymal transition interactome gene-expression signature is associated with claudin-low and metaplastic breast cancer subtypes. Proc Natl Acad Sci U S A. 2010 Aug 31;107(35):15449-54. (*Co-first author)

Komurov K, Padron D, Cheng T, Roth M, Rosenblatt KP, White MA. Comprehensive mapping of the human kinome to epidermal growth factor receptor signaling. J Biol Chem. 2010 Jul 2;285(27):21134-42.

Komurov K, Gunes MH, White MA. Fine-scale dissection of functional protein network organization by statistical network analysis. PLoS One. 2009 Jun 24;4(6):e6017.

Komurov K, White M. Revealing static and dynamic modular architecture of the eukaryotic protein interaction network. Mol Syst Biol. 2007;3:110.

Arumugam P, Malik P. Genetic therapy for beta-thalassemia: from the bench to the bedside.Hematology Am Soc Hematol Educ Program. 2010;2010:445-50.

Perumbeti A, Malik P. Therapy for beta-globinopathies: a brief review and determinants for successful and safe correction.Ann N Y Acad Sci. 2010 Aug;1202:36-44. Review.

Perumbeti A, Malik P. Genetic correction of sickle cell anemia and beta-thalassemia: progress and new perspective.ScientificWorldJournal. 2010 Apr 13;10:644-54. Review.

Sundaram N, Tailor A, Mendelsohn L, Wansapura J, Wang X, Higashimoto T, Pauciulo MW, Gottliebson W, Kalra VK, Nichols WC, Kato GJ, Malik P. High levels of placenta growth factor in sickle cell disease promote pulmonary hypertension.Blood. 2010 Jul 8;116(1):109-12.

Wang D, Zhang W, Kalfa TA, Grabowski G, Davies S, Malik P, Pan D. Reprogramming erythroid cells for lysosomal enzyme production leads to visceral and CNS cross-correction in mice with Hurler syndrome.Proc Natl Acad Sci U S A. 2009 Nov 24;106(47):19958-63.

Arumugam PI, Urbinati F, Velu CS, Higashimoto T, Grimes HL, Malik P. The 3' region of the chicken hypersensitive site-4 insulator has properties similar to its core and is required for full insulator activity.PLoS One. 2009 Sep 10;4(9):e6995.

Arumugam PI, Higashimoto T, Urbinati F, Modlich U, Nestheide S, Xia P, Fox C, Corsinotti A, Baum C, Malik P. Genotoxic potential of lineage-specific lentivirus vectors carrying the beta-globin locus control region.Mol Ther. 2009 Nov;17(11):1929-37.

Perumbeti A, Higashimoto T, Urbinati F, Franco R, Meiselman HJ, Witte D, Malik P. A novel human gamma-globin gene vector for genetic correction of sickle cell anemia in a humanized sickle mouse model: critical determinants for successful correction.Blood. 2009 Aug 6;114(6):1174-85.

Urbinati F, Arumugam P, Higashimoto T, Perumbeti A, Mitts K, Xia P, Malik P. Mechanism of reduction in titers from lentivirus vectors carrying large inserts in the 3'LTR.Mol Ther. 2009 Sep;17(9):1527-36.

Williams JP, Wu J, Johansson G, Rizvi TA, Miller SC, Geiger H, Malik P, Li W, Mukouyama YS, Cancelas JA, Ratner N. Nf1 mutation expands an EGFR-dependent peripheral nerve progenitor that confers neurofibroma tumorigenic potential.Cell Stem Cell. 2008 Dec 4;3(6):658-69.

Clinical Interests

Fanconi anemia; chromosome instability; DNA repair; multiprotein complex

Research Interests

Research in my laboratory focuses on functional analysis of Fanconi anemia gene products. The major research focus includes identification of new FA genes and signal transduction pathways that regulate DNA damage induced activation of the FA-core complex. Important technologies include biochemical purification of multiprotein complexes from human cell extracts, immunoprecipitation, RNAi, and biochemical assays. My long-term research goal is to use Fanconi anemia as a model system to study some of the important fundamental questions of cancer biology in general.

Singh TR, Saro D, Ali AM, Zheng XF, Du CH, Killen MW, Sachpatzidis A, Wahengbam K, Pierce AJ, Xiong Y, Sung P, Meetei AR. MHF1-MHF2, a histone-fold-containing protein complex, participates in the Fanconi anemia pathway via FANCM.Mol Cell. 2010 Mar 26;37(6):879-86.

Singh TR, Bakker ST, Agarwal S, Jansen M, Grassman E, Godthelp BC, Ali AM, Du CH, Rooimans MA, Fan Q, Wahengbam K, Steltenpool J, Andreassen PR, Williams DA, Joenje H, de Winter JP, Meetei AR. Impaired FANCD2 monoubiquitination and hypersensitivity to camptothecin uniquely characterize Fanconi anemia complementation group M.Blood. 2009 Jul 2;114(1):174-80.

Ali AM, Kirby M, Jansen M, Lach FP, Schulte J, Singh TR, Batish SD, Auerbach AD, Williams DA, Meetei AR. Identification and characterization of mutations in FANCL gene: a second case of Fanconi anemia belonging to FA-L complementation group.Hum Mutat. 2009 Jul;30(7):E761-70.

Ali AM, Singh TR, Meetei AR. FANCM-FAAP24 and FANCJ: FA proteins that metabolize DNA.Mutat Res. 2009 Jul 31;668(1-2):20-6. Epub 2009 Apr 18. Review.

Singh TR, Ali AM, Busygina V, Raynard S, Fan Q, Du CH, Andreassen PR, Sung P, Meetei AR. BLAP18/RMI2, a novel OB-fold-containing protein, is an essential component of the Bloom helicase-double Holliday junction dissolvasome.Genes Dev. 2008 Oct 15;22(20):2856-68.

Meetei AR, Medhurst AL, Ling C, Xue Y, Singh TR, Bier P, Steltenpool J, Stone S, Dokal I, Mathew CG, Hoatlin M, Joenje H, de Winter JP, Wang W. A human ortholog of archaeal DNA repair protein Hef is defective in Fanconi anemia complementation group M.Nat Genet. 2005 Sep;37(9):958-63.

Mizukawa B, Wei J, Shrestha M, Wunderlich M, Chou FS, Griesinger A, Harris CE, Kumar AR, Zheng Y, Williams DA, Mulloy JC. Inhibition of Rac GTPase signaling and downstream prosurvival Bcl-2 proteins as combination targeted therapy in MLL-AF9 leukemia. Blood. 2011 Nov 10;118(19):5235-45.

Chou FS, Wunderlich M, Griesinger A, Mulloy JC. N-RasG12D induces features of stepwise transformation in preleukemic human umbilical cord blood cultures expressing the AML1-ETO fusion gene.Blood. 2011 Feb 17;117(7):2237-40.

Mulloy JC. Peripheral T cell lymphoma: new model + new insight.J Exp Med. 2010 May 10;207(5):911-3.

Mulloy JC, Cancelas JA, Filippi MD, Kalfa TA, Guo F, Zheng Y. Rho GTPases in hematopoiesis and hemopathies.Blood. 2010 Feb 4;115(5):936-47.

Link KA, Chou FS, Mulloy JC. Core binding factor at the crossroads: determining the fate of the HSC.J Cell Physiol. 2010 Jan;222(1):50-6. Review.

Wunderlich M, Mulloy JC. Model systems for examining effects of leukemia-associated oncogenes in primary human CD34+ cells via retroviral transduction. Methods Mol Biol. 2009;538:263-85.

Bosco EE, Mulloy JC, Zheng Y. Rac1 GTPase: a "Rac" of all trades.Cell Mol Life Sci. 2009 Feb;66(3):370-4. Review.

Mulloy JC, Wunderlich M, Zheng Y, Wei J. Transforming human blood stem and progenitor cells: a new way forward in leukemia modeling.Cell Cycle. 2008 Nov 1;7(21):3314-9.

Wei J, Wunderlich M, Fox C, Alvarez S, Cigudosa JC, Wilhelm JS, Zheng Y, Cancelas JA, Gu Y, Jansen M, Dimartino JF, Mulloy JC. Microenvironment determines lineage fate in a human model of MLL-AF9 leukemia.Cancer Cell. 2008 Jun;13(6):483-95.

Krejci O, Wunderlich M, Geiger H, Chou FS, Schleimer D, Jansen M, Andreassen PR, Mulloy JC. p53 signaling in response to increased DNA damage sensitizes AML1-ETO cells to stress-induced death.Blood. 2008 Feb 15;111(4):2190-9.

Jackoncic J, Sondgeroth B, Carpino N, Nassar N. “The 1.35 Å structure of the Phosphatase domain of the Suppressor of T Cell Receptor Signaling Protein in complex with Sulfate”.Acta Cryst section F66, 2010  643-647.

Nassar N, Singh K, Garcia-Diaz M. “Structure of the dominant negative S17N mutant of Ras” Biochemistry. 2010 49,1970-1974.

Ford B, Boykevisch S, Zhao C, Kunzelmann S, Bar-Sagi D, Herrmann C, Nassar N. “Characterization of a Ras mutant with identical GDP- and GTP-bound structures”. Biochemistry. 2009  48,11449-11457.

Carpino N, Chen Y, Nassar N, Hye-Won Oh. “The Sts proteins target tyrosine phosphorylated, ubiquitinated proteins within TCR signaling pathways”. in review. J. Immuno. 2009  46,3224-3231.

Chen Y, Jakoncic J, Parker KA, Carpino N, Nassar N. “Structures of the Phosphorylated and VO3-bound 2H-Phosphatase Domain of Sts-2”. Biochemistry. 2009  48, 8129-8135.

Chen Y, Jakoncic J, Carpino N, Nassar N. “Structural and Functional Characterization of the 2H-phosphatase domain of Sts-2 reveals an Acid-Dependent Phosphatase Activity”. Biochemistry. 2009  48,1681-1690.

Chen Y, Jakoncic J, Keller J, Wang J, Zheng X, Carpino N, Nassar N. "Structural and functional characterization of the C-terminal domain of the ecdysteroid phosphate phosphatase from Bombix mori" Biochemistry. 2008  47,12135-12145.

Mikhailik A, Ford B, Keller J, Chen Y, Nassar N, Carpino N. "The C-terminal mutase-like domain of Sts-1 is important for its T-cell suppressor activity". Molecular Cell. 2007  27, 486-487.

Hematopoietic stem cells; Mesenchymal stem/progenitor cells; Gene therapy; Human genetics; Translational research; Lysosomal storage diseases

Visit the Pan Lab.

Pan D*, Kalfa TA, Wang D, Risinger M, Crable S, Ottlinger A, Mount DB, Hubner CA, Franco RS, and Joiner CH*. KCl cotransporter gene expression during human and murine erythroid differentiation, J Biol Chem, 286(35): 30492-30503, 2011. co-correspondent authors.

Pan D. Cell- and Gene-Based Therapeutic Approaches for Neurological Deficits in Mucopolysaccharidoses.Curr Pharm Biotechnol. 2011 Jan 11.

Kalfa TA, Pushkaran S, Zhang X, Johnson JF, Pan D, Daria D, Geiger H, Cancelas JA, Williams DA, Zheng Y. Rac1 and Rac2 GTPases are necessary for early erythropoietic expansion in the bone marrow but not in the spleen.Haematologica. 2010 Jan;95(1):27-35.

Wang D, Zhang W, Kalfa TA, Grabowski G, Davies S, Malik P, Pan D. Reprogramming erythroid cells for lysosomal enzyme production leads to visceral and CNS cross-correction in mice with Hurler syndrome.Proc Natl Acad Sci U S A. 2009 Nov 24;106(47):19958-63.

Pan D. In situ (in vivo) gene transfer into murine bone marrow stem cells. Methods Mol Biol. 2009;506:159-69.

Wang D, Worsham DN, Pan D. Co-expression of MGMT(P140K) and alpha-L-iduronidase in primary hepatocytes from mucopolysaccharidosis type I mice enables efficient selection with metabolic correction.J Gene Med. 2008 Mar;10(3):249-59.

Pan D, Sciascia A 2nd, Vorhees CV, Williams MT. Progression of multiple behavioral deficits with various ages of onset in a murine model of Hurler syndrome.Brain Res. 2008 Jan 10;1188:241-53.

Worsham DN, Schuesler T, von Kalle C, Pan D. In vivo gene transfer into adult stem cells in unconditioned mice by in situ delivery of a lentiviral vector. Mol Ther. 2006 Oct;14(4):514-24.

Signal transduction in Fanconi anemia and its evolution to leukemia.

Visit the Pang Lab.

Du W, Li XE, Sipple J, Pang Q. Overexpression of IL-3R{alpha} on CD34+CD38- stem cells defines leukemia-initiating cells in Fanconi anemia AML. Blood. 2011 Apr 21;117(16):4243-52.

Du W, Li J, Sipple J, Chen J, Pang Q. Cytoplasmic FANCA-FANCC complex interacts and stabilizes the cytoplasm-dislocalized leukemic nucleophosmin protein (NPMc). J Biol Chem. 2010 Nov 26;285(48):37436-44.

Li J, Du W, Maynard S, Andreassen PR, Pang Q. Oxidative stress-specific interaction between FANCD2 and FOXO3a. Blood. 2010 Feb 25;115(8):1545-8.

Du W, Zhou Y, Pike S, Pang Q. NPM phosphorylation stimulates Cdk1, overrides G2/M checkpoint and increases leukemic blasts in mice. Carcinogenesis. 2010 Feb;31(2):302-10.

Pang Q, Andreassen PR. Fanconi anemia proteins and endogenous stresses. Mutat Res. 2009 Jul 31;668(1-2):42-53.

Rani R, Li J, Pang Q. Differential p53 engagement in response to oxidative and oncogenic stresses in Fanconi anemia mice. Cancer Res. 2008 Dec 1;68(23):9693-702.

Zhou Y, Du W, Koretsky T, Bagby GC, Pang Q. TAT-mediated intracellular delivery of NPM-derived peptide induces apoptosis in leukemic cells and suppresses leukemogenesis in mice. Blood. 2008 Sep 15;112(6):2474-83.

Zhang X, Shang X, Guo F, Murphy K, Kirby M, Kelly P, Reeves L, Smith FO, Williams DA, Zheng Y, Pang Q. Defective homing is associated with altered Cdc42 activity in cells from patients with Fanconi anemia group A. Blood. 2008 Sep 1;112(5):1683-6.

Li J, Sejas DP, Zhang X, Qiu Y, Nattamai KJ, Rani R, Rathbun KR, Geiger H, Williams DA, Bagby GC, Pang Q. TNF-alpha induces leukemic clonal evolution ex vivo in Fanconi anemia group C murine stem cells. J Clin Invest. 2007 Nov;117(11):3283-95.

Zhang X, Sejas DP, Qiu Y, Williams DA, Pang Q. Inflammatory ROS promote and cooperate with the Fanconi anemia mutation for hematopoietic senescence. J Cell Sci. 2007 May 1;120(Pt 9):1572-83.

Clinical Interests

Development of the nervous system; peripheral nerve tumor formation

Research Interests

Genetic mutations in tumor suppressor genes

Visit the Ratner Lab.

PhD: Indiana University, 1982.

BA: Brown University, 1975.

Fellowship: Washington University St. Louis, 1982-1987.

Mayes DA, Rizvi TA, Cancelas JA, Kolasinski NT, Ciraolo GM, Stemmer-Rachamimov AO, Ratner N. Perinatal or Adult Nf1 Inactivation Using Tamoxifen-Inducible PlpCre Each Cause Neurofibroma Formation. Cancer Res. 2011 Jul 1;71(13):4675-85.

Shang X, Cancelas JA, Li L, Guo F, Liu W, Johnson JF, Ficker A, Daria D, Geiger H, Ratner N, Zheng YR-Ras and Rac1 GTPase Crosstalk Regulates Hematopoietic Progenitor Cell Migration, Homing and Mobilization.Biol Chem. 2011 May 13.

Wu., J., Dombi, E.,Jousma, E., Dunn, S.R., Lindquist, D., Kim, M., Kim, A., Cripe, T.P., and Ratner, N. Magnetic resonance imaging to monitor effects of Sorafenib and RAD001 in the DhhCre;Nf1fl/fl mouse model of plexiform neurofibroma.Ped. Blood & Cancer. 2011.

Hummel, T.R., Jessen, W.J., Miller, S.C., Kluwe, L, Mautner, V.F., Wallace, M.R., Lázaro, C. Page, G., Worley, P., Aronow, B.J., Schorry, E., and Ratner, N. Gene expression analysis identifies potential biomarkers of neurofibromatosis type 1 including adrenomedullin. Clin Cancer Res. 2010;16 5048-5057.

Ryan, M.A., Nattamai, K.J., Xing, E., Schleimer, D., Daria, D., Sengupta, A., Köhler, A., Liu, W., Gunzer, M., Jansen, M., Ratner, N., Le Cras, T.D., Waterstrat, A., Van Zant,G., Cancelas, J.A., Zheng, Y., and Geiger, H. Pharmacological inhibition of EGFR signaling enhances G-CSF induced hematopoietic stem cell mobilization.Nature Med., 2010 16(10):1141-6. 2010.

Miller, S.J., Lan, Z., Hardiman, A., Wu, J., Kordich, J.J., Patmore, D., Hegde, R., Cripe, T.P., Cancelas, J., Collins M. and Ratner, N. Inhibition of Eyes Absent Homolog 4 expression induces malignant peripheral nerve sheath tumor necrosis.Oncogene. 29(3):368-79. 2009.

Miller, S.J., Jessen, W.J., Mehta, T., Hardiman, A., Sites, E., Kaiser, S., Jegga, A., Li, H., Upadhyaya, M.., Giovannini, M., Muir, D., Wallace, M.R., Lopez, E.,  Serra, E., Lazaro, C., Stemmer-Rachamimov, A., Page, G., Aronow, B.J. and Ratner, N.  Integrative genomic analyses of neurofibromatosis tumors identify SOX9 as biomarker and survival gene. EMBO Mol. Medicine. 1(4): 236-248. 2009.

Mahller, Y., Williams, J., Baird, W., Mitton, B., Grossheim, J., Saeki, Y., Cancelas, J., Ratner, N., and Cripe, T. Neuroblastoma cell lines contain pluripotent tumor initiating cells that are susceptible to a targeted oncolytic virus. PLoS ONE. 4(1):e4235, 1 – 10. 2009.

Williams, J.P., Wu, J., Johansson, G., Rizvi, T., Miller, S.C., Geiger, H., Malik, P., Li, W., Mukouyama, Y., Cancelas, J.A. and Ratner, N.  Nf1 mutation expands an EGFR-dependent peripheral nerve progenitor population that confers neurofibroma tumorigenic potential. Cell Stem Cell. 3(6):658-69. 2008.

Williams, J.P., Wu, J., Johansson, G., Rizvi, T., Miller, S.C., Geiger, H., Malik, P., Li, W., Mukouyama, Y., Cancelas, J.A. and Ratner, N.  Nf1 mutation expands an EGFR-dependent peripheral nerve progenitor population that confers neurofibroma tumorigenic potential. Cell Stem Cell. 3(6):658-69. 2008.

Johansson, G., Mahller, Y., Collins, M.H., Kim, M-O., Nobukuni, T., Perentesis, J.P., Cripe, T.P., Lane, H.A., Kozma, S., Thomas, G., Ratner, N. Effective In Vivo Targeting of the mTOR Pathway in Malignant Peripheral Nerve Sheath Tumor.Mol. Cancer Therapeutics. 7(5):1237-45. 2008.

Starczynowski DT, Vercauteren S, Sung S, Brooks-Wilson A, Lam WL, Karsan A. Copy number alterations at polymorphic loci may be acquired somatically in patients with myelodysplastic syndromes. Leuk Res. 2011 Apr;35(4):444-7.

Starczynowski DT, Kuchenbauer F, Wegrzyn J, Rouhi A, Petriv O, Hansen CL, Humphries RK, Karsan A. MicroRNA-146a disrupts hematopoietic differentiation and survival. Exp Hematol. 2011 Feb;39(2):167-178.e4.

Starczynowski DT, Morin R, McPherson A, Lam J, Chari R, Wegrzyn J, Kuchenbauer F, Hirst M, Tohyama K, Humphries RK, Lam WL, Marra M, Karsan A. Genome-wide identification of human microRNAs located in leukemia-associated genomic alterations. Blood. 2011 Jan 13;117(2):595-607.

Starczynowski DT, Kuchenbauer F, Wegrzyn J, Rouhi A, Petriv O, Hansen CL, Humphries RK, Karsan A. MicroRNA-146a disrupts hematopoietic differentiation and survival. Exp Hematol. 2011 Feb;39(2):167-178. 

Vercauteren SM, Sung S, Starczynowski DT, Lam WL, Bruyere H, Horsman DE, Tsang P, Leitch H, Karsan A. Array comparative genomic hybridization of peripheral blood granulocytes of patients with myelodysplastic syndrome detects karyotypic abnormalities. Am J Clin Pathol. 2010 Jul;134(1):119-26.

Starczynowski DT and A Karsan.Innate immune signaling in Myelodysplastic Syndromes. Hematology/Oncology Clinics of North America. 2010 Apr;24(2):343-359.

Starczynowski DT and A Karsan. Deregulation of innate immune signaling in myelodysplastic syndromes is associated with deletion of chromosome arm 5q. Cell Cycle. 2010 Mar;9(5):855-6.

Starczynowski DT, F Kuchenbauer, B Argiropoulos, S Sung, R Morin, A Muranyi, D Hogue, R Wells, M Marra, WL Lam, K Humphries, and A Karsan. Identification of miR-145 and miR-146a as microRNAs involved in the pathogenesis of 5q- syndrome. Nature Medicine. 2010Jan;16(1):49-58.

Starczynowski DT, S Vercauteren, S Sung, A Brooks-Wilson, J Spinelli, C Eaves, A Eaves, D Horsman, W Lam, A Karsan. 2008. High-resolution array comparative genomic hybridization of CD34+ cells from patients with low-risk myelodysplastic syndromes predicts overall and leukemia-free survival. Blood. 2010 Oct;112(8): 3412-3424.

Starczynowski DT, Trautmann H, Pott C, Harder L, Arnold N, Africa JA, Leeman JR, Siebert R, Gilmore TD. Mutation of an IKK phosphorylation site within the transactivation domain of REL in two patients with B-cell lymphoma enhances REL's in vitro transforming activity. Oncogene. 2007 Apr 26;26(19):2685-94.

Starczynowski DT, Reynolds JG, Gilmore TD. Mutations of tumor necrosis factor alpha-responsive serine residues within the C-terminal transactivation domain of human transcription factor REL enhance its in vitro transforming ability. Oncogene. 2005 Nov 10;24(49):7355-68.

Development, scale-up and clinical production of viral vectors and cellular products

Dr. van der Loo is the Director of the Cincinnati Children's Hospital Medical Center's Aseptic Processing Laboratories, Vector Production Facility and Research Viral Vector Core.

BS: Biology, State University of Utrecht, the Netherlands, 1983.

MS: Medical Biology, State University of Utrecht, the Netherlands, 1987.

Teaching degree: Biology, State University of Utrecht, Netherlands, 1989.

PhD: Hematology, Erasmus University Rotterdam, the Netherlands, 1995.

Postdoctoral Fellowship: Hematology, Howard Hughes Medical Institute, Indiana University, Indianapolis, IN, 1994-1998.

Jeong Y, Dolson DK, Waclaw RR, Matise MP, Sussel L, Campbell K, Kaestner KH, and Epstein DJ. Spatial and temporal requirements for sonic hedgehog in the regulation of thalamic interneuron identity. Development. 2011;138:531-541

Waclaw RR, Ehrman LA, Pierani A, Campbell K. Developmental origin of the neuronal subtypes that comprise the amygdalar fear circuit in the mouse. J Neurosci. 2010 May 19;30(20):6944-53. 

Xu Q, Guo L, Moore H, Waclaw RR, Campbell K, Anderson SA. Sonic Hedgehog confers ventral telencephalic progenitors with distinct cortical interneuron fates. Neuron. 2010 Feb;65(3):328-340.

Waclaw RR, Campbell K. Regional control of cortical lamination. Nat Neurosci. 2009 Oct;12(10):1211-2.

Waclaw RR, Wang B, Pei Z, Ehrman LA, Campbell K. Distinct temporal requirements for the homeobox gene Gsx2 in specifying striatal and olfactory bulb neuronal fates. Neuron. 2009 Aug 27;63(4):451-65.

Wang B, Waclaw RR, Allen ZJ 2nd, Guillemot F, Campbell K. Ascl1 is a required downstream effector of Gsx gene function in the embryonic mouse telencephalon. Neural Dev. 2009 Feb 10;4:5.

Allen ZJ 2nd, Waclaw RR, Colbert MC, Campbell K. Molecular identity of olfactory bulb interneurons: transcriptional codes of periglomerular neuron subtypes. J Mol Histol. 2007 Dec;38(6):517-25.

Chen L, Liao G, Waclaw RR, Burns KA, Linquist D, Campbell K, Zheng Y, Kuan CY. Rac1 controls the formation of midline commissures and the competency of tangential migration in ventral telencephalic neurons. J Neurosci. 2007 Apr 4;27(14):3884-93. 

Waclaw RR, Allen ZJ 2nd, Bell SM, Erdélyi F, Szabó G, Potter SS, Campbell K. The zinc finger transcription factor Sp8 regulates the generation and diversity of olfactory bulb interneurons. Neuron. 2006 Feb 16;49(4):503-16. 

Miller SJ, Lan ZD, Hardiman A, Wu J, Kordich JJ, Patmore DM, Hegde RS, Cripe TP, Cancelas JA, Collins MH, Ratner N. Inhibition of Eyes Absent Homolog 4 expression induces malignant peripheral nerve sheath tumor necrosis. Oncogene. 2010 Jan 21;29(3):368-79. 

Ren XP, Wu J, Wang X, Sartor MA, Qian J, Jones K, Nicolaou P, Pritchard TJ, Fan GC. MicroRNA-320 is involved in the regulation of cardiac ischemia/reperfusion injury by targeting heat-shock protein 20. Circulation. 2009 May 5;119(17):2357-66.

Kurth CD, McCann JC, Wu J, Miles L, Loepke AW. Cerebral oxygen saturation-time threshold for hypoxic-ischemic injury in piglets. Anesth Analg. 2009 Apr;108(4):1268-77. 

Williams JP, Wu J, Johansson G, Rizvi TA, Miller SC, Geiger H, Malik P, Li W, Mukouyama YS, Cancelas JA, Ratner N. Nf1 mutation expands an EGFR-dependent peripheral nerve progenitor that confers neurofibroma tumorigenic potential. Cell Stem Cell. 2008 Dec 4;3(6):658-69.

Wu J, Williams JP, Rizvi TA, Kordich JJ, Witte D, Meijer D, Stemmer-Rachamimov AO, Cancelas JA, Ratner N. Plexiform and dermal neurofibromas and pigmentation are caused by Nf1 loss in desert hedgehog-expressing cells. Cancer Cell. 2008 Feb;13(2):105-16. 

Wu J, Bohanan CS, Neumann JC, Lingrel JB. KLF2 transcription factor modulates blood vessel maturation through smooth muscle cell migration. J Biol Chem. 2008 Feb 15;283(7):3942-50. 

 Monk KR, Wu J, Williams JP, Finney BA, Fitzgerald ME, Filippi MD, Ratner N. Mast cells can contribute to axon-glial dissociation and fibrosis in peripheral nerve. Neuron Glia Biol. 2007 Aug;3(3):233-44.

Wu J. Deep sedation with intravenous infusion of combined propofol and ketamine during dressing changes and whirlpool bath in patients with severe epidermolysis bullosa. Paediatr Anaesth. 2007 Jun;17(6):592-6.

Nelson LA, McCann JC, Loepke AW, Wu J, Ben Dor B, Kurth CD. Development and validation of a multiwavelength spatial domain near-infrared oximeter to detect cerebral hypoxia-ischemia. J Biomed Opt. 2006 Nov-Dec;11(6):064022.

Research Interests

Using transgenic and gene targeted mouse models to study the physiological and pathological roles of Rho GTPases and their regulators in hematopoiesis, neurogenesis, lung cancer development and small intestinal stem cell regulations; mechanism based, rational design of small molecule inhibitors targeting Rho GTPase signaling modules and pathologic functions in cancer and blood diseases; mouse model studies of mTor signaling function in hematopoietic stem cells and small intestinal stem cells.

BS: Tsinghua University, Beijing, China, 1986.

MS: Cornell University, Ithaca, NY 1988.

PhD: Cornell University, Ithaca, NY, 1991.

Postdoctoral Fellow: Cornell University, Ithaca, NY, 1995.

Stengel K, Zheng Y. Cdc42 in oncogenic transformation, invasion, and tumorigenesis. Cell Signal. 2011 Sep;23(9):1415-23.

Akbar H, Shang X, Perveen R, Berryman M, Funk K, Johnson JF, Tandon NN, Zheng Y. Gene Targeting Implicates Cdc42 GTPase in GPVI and Non-GPVI Mediated Platelet Filopodia Formation, Secretion and Aggregation. PLoS One. 2011;6(7):e22117.

Liu W, Feng Y, Shang X, Zheng Y. Rho GTPases in hematopoietic stem/progenitor cell migration. Methods Mol Biol. 2011;750:307-19.

Shang X, Cancelas JA, Li L, Guo F, Liu W, Johnson JF, Ficker A, Daria D, Geiger H, Ratner N, Zheng Y. R-Ras and Rac GTPase cross-talk regulates hematopoietic progenitor cell migration, homing, and mobilization. J Biol Chem. 2011 Jul 8;286(27):24068-78.

Wang X, Zhang F, Chen F, Liu D, Zheng Y, Zhang Y, Dong C, Su B. MEKK3 regulates IFN-gamma production in T cells through the Rac1/2-dependent MAPK cascades. J Immunol. 2011 May 15;186(10):5791-800.

Katayama K, Melendez J, Baumann JM, Leslie JR, Chauhan BK, Nemkul N, Lang RA, Kuan CY, Zheng Y, Yoshida Y. Loss of RhoA in neural progenitor cells causes the disruption of adherens junctions and hyperproliferation. Proc Natl Acad Sci U S A. 2011 May 3;108(18):7607-12.

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