Nichols Lab

Publications

Tai, YY; Yu, Q; Tang, Y; Sun, W; Kelly, NJ; Okawa, S; Zhao, J; Schwantes-An, TH; Lacoux, C; Torrino, S; et al. Allele-specific control of rodent and human lncRNA KMT2E-AS1 promotes hypoxic endothelial pathology in pulmonary hypertension. Science Translational Medicine. 2024; 16:eadd2029.

Jose, A; Elwing, JM; Kawut, SM; Pauciulo, MW; Sherman, KE; Nichols, WC; Fallon, MB; McCormack, FX. Human liver single nuclear RNA sequencing implicates BMPR2, GDF15, arginine, and estrogen in portopulmonary hypertension. Communications Biology. 2023; 6:826.

Welch, CL; Aldred, MA; Balachandar, S; Dooijes, D; Eichstaedt, CA; Gräf, S; Houweling, AC; Machado, RD; Pandya, D; Prapa, M; et al. Defining the clinical validity of genes reported to cause pulmonary arterial hypertension. Genetics in Medicine. 2023; 25:100925.

Alotaibi, M; Liu, Y; Magalang, GA; Kwan, AC; Ebinger, JE; Nichols, WC; Pauciulo, MW; Jain, M; Cheng, S. Deriving Convergent and Divergent Metabolomic Correlates of Pulmonary Arterial Hypertension. Metabolites. 2023; 13:802.

Torres, G; Lancaster, AC; Yang, J; Griffiths, M; Brandal, S; Damico, R; Vaidya, D; Simpson, CE; Martin, LJ; Pauciulo, MW; et al. Low-affinity insulin-like growth factor binding protein 7 and its association with pulmonary arterial hypertension severity and survival. Pulmonary Circulation. 2023; 13:e12284.

Ortega, R; Burbulla, L; Mileva, I; Taylor, G; Cuperlovic-Culf, M; Glickman, A; Pullman, M; Scherzer, C; Raymond, D; Miltenyi-Miltenberger, G; et al. Peripheral Sphingolipids as Potential Biomarkers of Parkinson disease Including Sex-Related Differences (P3-11.007). Neurology. 2023; 100.

Sangam, S; Sun, X; Schwantes-An, TH; Yegambaram, M; Lu, Q; Shi, Y; Cook, T; Fisher, A; Frump, AL; Coleman, A; et al. SOX17 Deficiency Mediates Pulmonary Hypertension: At the Crossroads of Sex, Metabolism, and Genetics. American Journal of Respiratory and Critical Care Medicine. 2023; 207:1055-1069.

Torres, G; Yang, J; Griffiths, M; Brandal, S; Damico, R; Vaidya, D; Simpson, CE; Pauciulo, MW; Nichols, WC; Ivy, DD; et al. Insulin-like growth factor binding Protein-4: A novel indicator of pulmonary arterial hypertension severity and survival. Pulmonary Circulation. 2023; 13:e12235.

Prohaska, CC; Zhang, X; Schwantes-An, TH L; Stearman, RS; Hooker, S; Kittles, RA; Aldred, MA; Lutz, KA; Pauciulo, MW; Nichols, WC; et al. RASA3 is a candidate gene in sickle cell disease-associated pulmonary hypertension and pulmonary arterial hypertension. Pulmonary Circulation. 2023; 13:e12227.

Eichstaedt, CA; Belge, C; Chung, WK; Gräf, S; Grünig, E; Montani, D; Quarck, R; Tenorio-Castano, JA; Soubrier, F; Trembath, RC; et al. Genetic counselling and testing in pulmonary arterial hypertension: a consensus statement on behalf of the International Consortium for Genetic Studies in PAH. The European respiratory journal : official journal of the European Society for Clinical Respiratory Physiology. 2023; 61:2201471.

Hong, J; Wong, B; Rhodes, CJ; Kurt, Z; Schwantes-An, T; Mickler, EA; Gräf, S; Eyries, M; Lutz, KA; Pauciulo, MW; et al. Integrative Multiomics to Dissect the Lung Transcriptional Landscape of Pulmonary Arterial Hypertension. 2023; 4:2023.01.12.523812.

Alotaibi, M; Shao, J; Pauciulo, MW; Nichols, WC; Hemnes, AR; Malhotra, A; Kim, NH; Yuan, JX J; Fernandes, T; Kerr, KM; et al. Metabolomic Profiles Differentiate Scleroderma-PAH From Idiopathic PAH and Correspond With Worsened Functional Capacity. Chest. 2023; 163:204-215.

Harbaum, L; Rhodes, CJ; Wharton, J; Lawrie, A; Karnes, JH; Desai, AA; Nichols, WC; Humbert, M; Montani, D; Girerd, B; et al. Mining the Plasma Proteome for Insights into the Molecular Pathology of Pulmonary Arterial Hypertension. American Journal of Respiratory and Critical Care Medicine. 2022; 205:1449-1460.

Simpson, CE; Griffiths, M; Yang, J; Nies, MK; Vaidya, D; Brandal, S; Martin, LJ; Pauciulo, MW; Lutz, KA; Coleman, AW; et al. COL18A1 genotypic associations with endostatin levels and clinical features in pulmonary arterial hypertension: a quantitative trait association study. ERJ Open Research. 2022; 8:725-2021.

Toshner, M; Church, C; Harbaum, L; Rhodes, C; Villar Moreschi, SS; Liley, J; Jones, R; Arora, A; Batai, K; Desai, AA; et al. Mendelian randomisation and experimental medicine approaches to interleukin-6 as a drug target in pulmonary arterial hypertension. The European respiratory journal : official journal of the European Society for Clinical Respiratory Physiology. 2022; 59:2002463.

Pal, G; Mangone, G; Hill, EJ; Ouyang, B; Liu, Y; Lythe, V; Ehrlich, D; Saunders-Pullman, R; Shanker, V; Bressman, S; et al. Parkinson Disease and Subthalamic Nucleus Deep Brain Stimulation: Cognitive Effects in GBA Mutation Carriers. Annals of Neurology. 2022; 91:424-435.

Griffiths, M; Yang, J; Vaidya, D; Nies, M; Brandal, S; Ivy, DD; Hickey, F; Wolter-Warmerdam, K; Austin, ED; Mullen, M; et al. Biomarkers of Pulmonary Hypertension Are Altered in Children with Down Syndrome and Pulmonary Hypertension. The Journal of Pediatrics. 2022; 241:68-76.e3.

Yang, J; Ambade, AS; Nies, M; Griffiths, M; Damico, R; Vaidya, D; Brandal, S; Pauciulo, MW; Lutz, KA; Coleman, AW; et al. Hepatoma-derived growth factor is associated with pulmonary vascular remodeling and PAH disease severity and survival. Pulmonary Circulation. 2022; 12:e12007.

Zhu, N; Swietlik, EM; Welch, CL; Pauciulo, MW; Hagen, JJ; Zhou, X; Guo, Y; Karten, J; Pandya, D; Tilly, T; et al. Rare variant analysis of 4241 pulmonary arterial hypertension cases from an international consortium implicates FBLN2, PDGFD, and rare de novo variants in PAH. Genome Medicine: medicine in the post-genomic era. 2021; 13:80.

Daly, CM; Griffiths, M; Simpson, CE; Yang, J; Damico, RL; Vaidya, RD; Williams, M; Brandal, S; Jone, PN; Polsen, C; et al. Angiostatic Peptide, Endostatin, Predicts Severity in Pediatric Congenital Heart Disease-Associated Pulmonary Hypertension. Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease. 2021; 10:e021409.

Simpson, CE; Griffiths, M; Yang, J; Nies, MK; Vaidya, RD; Brandal, S; Martin, LJ; Pauciulo, MW; Lutz, KA; Coleman, AW; et al. The angiostatic peptide endostatin enhances mortality risk prediction in pulmonary arterial hypertension. ERJ Open Research. 2021; 7:378-2021.

David, FJ; Munoz, MJ; Shils, JL; Pauciulo, MW; Hale, PT; Nichols, WC; Afshari, M; Sani, S; Verhagen Metman, L; Corcos, DM; et al. Subthalamic Peak Beta Ratio Is Asymmetric in Glucocerebrosidase Mutation Carriers With Parkinson's Disease: A Pilot Study. Frontiers in Neurology. 2021; 12:723476.

Griffiths, M; Yang, J; Nies, M; Damico, RL; Ivy, DD; Martin, W; Nichols, B; Pauciulo, M; Everett, AD. (2021) ST2 Is a Biomarker of Pediatric Pulmonary Arterial Hypertension Severity and Clinical Worsening. Elsevier BV. 160:297-306.

Oldham, WM; Hemnes, AR; Aldred, MA; Barnard, J; Brittain, EL; Chan, SY; Cheng, F; Cho, MH; Desai, AA; Garcia, JG N; et al. NHLBI-CMREF Workshop Report on Pulmonary Vascular Disease Classification: JACC State-of-the-Art Review. Journal of the American College of Cardiology. 2021; 77:2040-2052.

Moran, EE; Bressman, SB; Ortega, RA; Raymond, D; Nichols, WC; Palmese, CA; Elango, S; Swan, M; Shanker, V; Perera, I; et al. Cognitive Functioning of Glucocerebrosidase (GBA) Non-manifesting Carriers. Frontiers in Neurology. 2021; 12:635958.

Swietlik, EM; Greene, D; Zhu, N; Megy, K; Cogliano, M; Rajaram, S; Pandya, D; Tilly, T; Lutz, KA; Welch, CC L; et al. Bayesian Inference Associates Rare KDR Variants with Specific Phenotypes in Pulmonary Arterial Hypertension. Circulation: Genomic and Precision Medicine. 2021; 14:e003155.

Badlam, JB; Badesch, DB; Austin, ED; Benza, RL; Chung, WK; Farber, HW; Feldkircher, K; Frost, AE; Poms, AD; Lutz, KA; et al. United States Pulmonary Hypertension Scientific Registry: Baseline Characteristics. Chest. 2021; 159:311-327.

Griffiths, M; Yang, J; Nies, M; Vaidya, D; Brandal, S; Williams, M; Matsui, EC; Grant, T; Damico, R; Ivy, D; et al. Pediatric pulmonary hypertension: insulin-like growth factor-binding protein 2 is a novel marker associated with disease severity and survival. Pediatric Research. 2020; 88:850-856.

Yang, J; Griffiths, M; Nies, MK; Brandal, S; Damico, R; Vaidya, D; Tao, X; Simpson, CE; Kolb, TM; Mathai, SC; et al. Insulin-like growth factor binding protein-2: a new circulating indicator of pulmonary arterial hypertension severity and survival. BMC Medicine. 2020; 18:268.

Rhodes, CJ; Otero-Nunez, P; Wharton, J; Swietlik, EM; Kariotis, S; Harbaum, L; Dunning, MJ; Elinoff, JM; Errington, N; Thompson, AA R; et al. Whole-Blood RNA Profiles Associated with Pulmonary Arterial Hypertension and Clinical Outcome. American Journal of Respiratory and Critical Care Medicine. 2020; 202:586-594.

Chen, JY; Griffiths, M; Yang, J; Nies, MK; Damico, RL; Simpson, CE; Vaidya, RD; Brandal, S; Ivy, DD; Austin, ED; et al. Elevated Interleukin-6 Levels Predict Clinical Worsening in Pediatric Pulmonary Arterial Hypertension. The Journal of Pediatrics. 2020; 223:164-169.e1.

Oriaku, I; LeSieur, MN; Nichols, WC; Barrios, R; Elliott, CG; Frost, A. A novel BMPR2 mutation with widely disparate heritable pulmonary arterial hypertension clinical phenotype. Pulmonary Circulation. 2020; 10:2045894020931315.

Potus, F; Pauciulo, MW; Cook, EK; Zhu, N; Hsieh, A; Welch, CL; Shen, Y; Tian, L; Lima, P; Mewburn, J; et al. Novel Mutations and Decreased Expression of the Epigenetic Regulator TET2 in Pulmonary Arterial Hypertension. Circulation. 2020; 141:1986-2000.

Karnes, JH; Wiener, HW; Wiener, H; Schwantes-An, T; Natarajan, B; Sweatt, AJ; Chaturvedi, A; Chaturvedi, A; Arora, AH; Batai, K; et al. Genetic Admixture and Survival in Diverse Populations with Pulmonary Arterial Hypertension. American Journal of Respiratory and Critical Care Medicine. 2020; 201:1407-1415.

Simpson, CE; Damico, RL; Hassoun, PM; Martin, LJ; Yang, J; Nies, MK; Vaidya, RD; Brandal, S; Pauciulo, MW; Austin, ED; et al. Noninvasive Prognostic Biomarkers for Left-Sided Heart Failure as Predictors of Survival in Pulmonary Arterial Hypertension. Chest. 2020; 157:1606-1616.

Simpson, CE; Chen, JY; Damico, RL; Hassoun, PM; Martin, LJ; Yang, J; Nies, M; Griffiths, M; Vaidya, RD; Brandal, S; et al. Cellular sources of interleukin-6 and associations with clinical phenotypes and outcomes in pulmonary arterial hypertension. The European respiratory journal : official journal of the European Society for Clinical Respiratory Physiology. 2020; 55:1901761.

Ulrich, A; Wharton, J; Thayer, TE; Swietlik, EM; Assad, TR; Desai, AA; Graf, S; Harbaum, L; Humbert, M; Morrell, NW; et al. Mendelian randomisation analysis of red cell distribution width in pulmonary arterial hypertension. The European respiratory journal : official journal of the European Society for Clinical Respiratory Physiology. 2020; 55:1901486.