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My lab reflects both my interests as a scientist and as a pediatric cardiologist. I investigate the pathology behind abnormal heart function, and I am interested in uncovering the mechanisms that help the myocardium adapt to compensate for these abnormalities. One particular subject I study is the misfolded protein response, a mechanism by which the body targets abnormal cellular proteins for removal. In collaboration with Jeffrey Robbins, PhD, and other members of his laboratory, I am exploring the consequences of preamyloid oligomer accumulation (a manifestation of the misfolded protein response) in transgenic mouse models and human myocardium. In this venue, I am actively involved in the training of students and pediatric cardiology fellows.
My second broad area of interest is echocardiography in research animals. As director of the Mouse Echocardiography Core, I work with research sonographers to obtain detailed anatomic and functional data for a variety of species. We use a Vevo 770 ultrasound system equipped with high-frequency transducers, a tool optimized for small animal imaging. With this system we are able to acquire highly detailed two-dimensional images, as well as M-mode and Doppler images, of the heart and surrounding vascular structures. This imaging ability provides a core service to a number of investigators at Cincinnati Children’s.
Cheek J, Wirrig E, Alfieri C, James J and Yutzey K. Differential activation of valvulogenic, chondrogenic and osteogenic pathways in mouse models of myxomatous and calcific aortic valve disease. J Mol Cell Cardiol. 52(3):689-700. 2012. PMCID: PMC3294059
Sadayappan S, Gulick J, Martin L, Osinska H, Barefield D, Cuello F, Avkiran M, Lasko V, Lorenz J, Maillet M, Martin J, Heller-Brown J, Bers D, Molkentin J, James J and Robbins J. A critical function for Ser-282 in cardiac myosin binding protein-C phosphorylation and cardiac function. Circ Res. 109(2):141-50. 2011. PMCID: PMC3132348
James J, Kinnett K, Ittenbach R, Wang Y, Benson D and Cripe L. Electrocardiographic abnormalities in very young Duchenne muscular dystrophy patients precede the onset of cardiac dysfunction. Neuromuscul Disord. 21(7):462-7. 2011. PMID: 21571532.
Combs M, Braitsch C, Lange A, James J and Yutzey K. NFATc1 promotes epicardium-derived cell (EPDC) invasion into myocardium. Development. 138(9):1747-57. 2011. PMCID: PMC3074451
James J and Robbins J. Signaling and myosin binding protein C. J Biol Chem. 286(12):9913-9. 2011. PMCID: PMC3060544
Stanley BA, Graham DR, James J, Mitsak M, Tarwater PM, Robbins J, Van Eyk JE. Altered myofilament stoichiometry in response to heart failure in a cardioprotective a-myosin heavy chain transgenic rabbit model. Proteomics Clin Appl. 5(3-4):147-58. 2011. PMCID: PMC3124290
Hinton R, Adelman-Brown J, Witt S, Krishnamurthy V, Gruber M, Osinska H, Sakthivel B, James J, Narmoneva D, Mecham R, Benson D. Elastin haploinsufficiency results in progressive aortic valve malformation and latent valve disease in a mouse model. Circ Res. 107(4):549-57. 2010.
James J, Hor K, Moga M, Martin L, Robbins J. Effects of myosin heavy chain manipulation in experimental heart failure. J Mol Cell Cardiol. 48(5):999-1006. May, 2010.
Kruger M, Zittrich S, Redwood C, Blaudeck N, James J, Robbins J, Pfitzer G, Stehle R. Effects of the mutation R145G in humancardiac troponin I on the kinetics of the contraction-relaxation cycle inisolated cardiac myofibrils. J. Physiol. 564:347-357.2005.
James J, Martin L, Krenz M, Quatman C, Jones F, Klevitsky R, Gulick J, Robbins J. Forced expression of alpha-myosin heavy chain in the rabbit ventricle results in cardioprotection under cardiomyopathic conditions. Circulation. 111:2339-2346. 2005.
Sanbe A, James J, Tuzcu V, Martin L, Gulick J, Osinska H, Sakthivel S, Ginsburg K, Bers D, Ziman B, Lakatta E, Robbins J. Transgenic rabbit model for human troponin I-based hypertrophic cardiomyopathy. Circulation. 111:2330-38. 2005.
Bhuijan M, Pattison J, James J, Gulick J, Osinska H and Robbins J. (2012) Atg7-dependent regulation of p62 in mice ameliorates desmin related cardiomyopathy. American Heart Association Scientific Sessions, Los Angeles, CA, November 2012.
Omar K, Hinton R, Jones H, Balaji S, Kim Y, Kim K, Leung A, Keswani S, James J, Crombleholme T and Habli M. (2012) Intra-placental gene transfer of human insulin-like growth factor-1 rescues postnatal intra-uterine growth restriction induced cardiac dysfunction. American Heart Association Scientific Sessions, Los Angeles, CA, November 2012.
Jain R, Martherus R, Takagi K, James J, Kramer K, Purevjav E and Towbin J. (2012) Effect of endurance exercise on transgenic arrhythmogenic right ventricular cardiomyopathy mice. American Heart Association Scientific Sessions, Los Angeles, CA, November 2012.
Huby A, Takagi K, Gong N, James J, Kramer K, Martherus R, Khuchua Z, Towbin J and Purevjav E. (2012) Restrictive cardiomyopathy due to myopalladin nonsense Q529X mutation is associated with ERK1/2 and CARP down-regulation. American Heart Association Scientific Sessions, Los Angeles, CA, November 2012.
Huby A, Takagi K, Gong H, James J, Martherus R, Kramer K, Khuchua A, Towbin J and Purevjav E. (2012) Exploring novel molecular mechanisms of restrictive cardiomyopathy. Heart Failure Society of American, Seattle, WA, September 2012.
Bhuiyan S, Pattison S, James J, Gulick J, Osinska H and Robbins J. (2012) Induced basal autophay in Atg7 mice ameliorates cardiac proteinopathy. BCVS 2012 Scientific Sessions, New Orleans, LA, July 2012.
Razzaque, A, Gulick J, Osinska H, Gupta M, James J and Robbins J. (2012) Pathogenicity of naturally-produced fragments of cardiac myosin binding protein C and their effects on heart function and failure. BCVS 2012 Scientific Sessions, New Orleans, LA, July 2012.
Gupta M, Gulick J, Osinska H, James J, Lasko V, Nieman M, Lorenz J and Robbins J. (2012) Functional dissection of myosin binding protein C phosphorylation. BCVS 2012 Scientific Sessions, New Orleans, LA, July 2012.
Garrison A, James J, Hinton R, Towbin J, Miller E, Parrott A, Goldenberg P, Jefferies J and Ware S. (2012) The utility of genetic testing in evaluation for Marfan syndrome. Platform presentation, American College of Medical Genetics, Charlotte, NC, March 2012.
Schulte C, Moore Victoria, Eghtesady P and James J. (2011) Fetal rat echocardiography: methods and normal values. American Society of Echocardiography, Montreal, Quebec, Canada, June 2011.
Cheek J, Wirrig E, Alfieri C, James J and Yutzey K. (2011) Differential activation of valve and bone developmental pathways in mouse models of valve disease. Weinstein Developmental Cardiology Conference, Cincinnati, OH, May 2011.
Huby A, James J, Purevjav E and Towbin J. (2011) Fas-ligand is a key player in development of apoptosis-induced cardiomyopathy in viral infection. Pediatric Academic Societies, Denver, CO, May 2011.
James J, Kinnett K, Wang Y, Ittenbach R and Cripe L. (2010) Electrocardiographic abnormalities in Duchenne muscular dystrophy prior to the onset of cardiac dysfunction. 15th International Congress of the World Muscle Society, Kumamoto, Japan, October 2010.
Cripe L, Kinnett K, Ittenbach R and James J. (2010) Prevalence of Sinus Tachycardia in Duchenne Muscular Dystrophy. 15th International Congress of the World Muscle Society, Kumamoto, Japan, October 2010.
James J, Osinska H, Malloy C, Margulies K and Robbins J. (2010) Accumulation of pre-amyloid oligomer in failing left ventricular myocardium. Keystone Symposium: Cardiovascular Development and Repair, Keystone, CO March 2010.
Cripe L, Kinnett K, Ittenbach R and James J. (2010) Prevalence of Sinus Tachycardia in Duchenne Muscular Dystrophy. Children’s Hospital of Philadelphia 13th Annual Update of Pediatric Cardiovascular Disease, Lake Buena Vista, FL, February 2010.
James J, Moga MA, Robbins J. Effect of Myosin Heavy Chain Composition on Global Cardiac Function in Pressure Overload Hypertrophy. International Society for Heart Research, June, 2008; Cincinnati, OH.
Cripe L, James J, Spicer R, Kinnett K, Markham L. Continuous intravenous milrinone infusion as therapy for end-stage heart failure in patients with Duchenne muscular dystrophy. Scientific Basis of Heart Failure in Children, May, 2008; Estes Park, CO.
James J, Markham L, Kinnett K, Benson D, Spicer R,Cripe L. Prevalence of ECG abnormalities in very young Duchenne muscular dystrophy patients. Scientific Basis of Heart Failure in Children, May 2008; Estes Park, CO.
Jeanne James, MD
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