Joyce Repa, Ph.D. Associate Professor School Medical School Department Physiology | Internal Medicine Graduate Programs Cell and Molecular Biology Biography Dr. Repa’s research at UT Southwestern focuses on the effects of diet and drugs on nuclear receptor regulation of gene expression, and how these signaling pathways contribute to such diseases as atherosclerosis,Type II diabetes, and the neurodegenerative disorder Niemann-Pick Type C (NPC). Her research is aimed at understanding the molecular mechanisms by which nuclear hormone receptors regulate lipid and carbohydrate metabolism in the liver, intestine, pancreatic islet, and central nervous system. Integrative approaches (transcriptomics, lipidomics), unique mouse strains, physiologic methods, and cell-based assays provide a comprehensive analysis of nuclear receptor pathways involved in disease progression and therapy. With the recent findings that hearing loss is an early consequence of NPC disease, and also results with cyclodextrin therapy for NPC, work is underway to determine the localization and role(s) of nuclear receptors and lipids on cochlear function. Education Undergraduate Uni of Wisconsin-Madison (1979), Biochemistry Graduate School Uni of Wisconsin-Madison (1990), Nutrition Graduate School Uni of Wisconsin-Madison (1990), Nutritional Sciences Graduate School Uni of Wisconsin-Madison (1996), Nutrition Research Interest Bile acids, Cholesterol Diabetes Lipidomics LXR, FXR, PXR, beta-cell, islet Neurodegenerative disease Nuclear hormone receptor Sensorineural hearing loss Transcription Publications Featured Publications Histone Deacetylase 9 Represses Cholesterol Efflux and Generation of Alternatively Activated Macrophages in Atherosclerosis Development. Cao Q, Rong S, Repa JJ, Clair RS, Parks JS, Mishra N Arterioscler. Thromb. Vasc. Biol. 2014 Jul Sustained and selective suppression of intestinal cholesterol synthesis by Ro 48-8071, an inhibitor of 2,3-oxidosqualene:lanosterol cyclase, in the BALB/c mouse. Chuang JC, Valasek MA, Lopez AM, Posey KS, Repa JJ, Turley SD Biochem. Pharmacol. 2014 Jan Hearing loss is an early consequence of NPC1 gene deletion in the mouse model of Niemann-Pick Disease, Type C. King KA, Gordon-Salant S, Pawlowski KS, Taylor AM, Griffith AJ, Houser A, Kurima K, Wassif CA, Wright CG, Porter FD, Repa JJ, Brewer CC J. Assoc. Res. Otolaryngol. 2014 15 4 529-541 A functional, genome-wide evaluation of liposensitive yeast identifies the ARE2 Required for Viability (ARV1) gene product as a major component of eukaryotic fatty acid resistance. Ruggles KV, Garbarino J, Liu Y, Moon J, Schneider K, Henneberry A, Billheimer J, Millar JS, Marchadier D, Valasek MA, Joblin-Mills A, Gulati S, Munkacsi AB, Repa JJ, Rader D, Sturley SL J. Biol. Chem. 2014 14 289 4417-4431 Characterization of Cholesterol Homeostasis in Telomerase-Immortalized Tangier Disease Fibroblasts Reveals Marked Phenotype Variability. Kannenberg F, Gorzelniak K, Jager K, Fobker M, Rust S, Repa J, Roth M, Bjorkhem I, Walter M J. Biol. Chem. 2013 Nov Role of Calcium and EPAC in Norepinephrine-induced Ghrelin Secretion. Mani BK, Chuang JC, Kjalarsdottir L, Sakata I, Walker AK, Kuperman A, Osborne-Lawrence S, Repa JJ, Zigman JM Endocrinology 2013 Nov Carboxylesterases are uniquely expressed among tissues and regulated by nuclear hormone receptors in the mouse. Jones RD, Taylor AM, Tong EY, Repa JJ Drug Metab. Dispos. 2013 Jan 41 1 40-9 Cyclodextrin mediates rapid changes in lipid balance in Npc1-/- mice without carrying cholesterol through the bloodstream. Taylor AM, Liu B, Mari Y, Liu B, Repa JJ J. Lipid Res. 2012 Nov 53 11 2331-42 Ghrelin Directly Stimulates Glucagon Secretion from Pancreatic {alpha}-Cells. Chuang JC, Sakata I, Kohno D, Perello M, Osborne-Lawrence S, Repa JJ, Zigman JM Molecular endocrinology (Baltimore, Md.) 2011 Jun 1600-11 Cyclodextrin overcomes the transport defect in nearly every organ of NPC1 mice leading to excretion of sequestered cholesterol as bile acid. Liu B, Ramirez CM, Miller AM, Repa JJ, Turley SD, Dietschy JM J. Lipid Res. 2010 May 51 5 933-44 Results 1-10 of 40 1 2 3 4 Next Last Honors & Awards J. Denis McGarry Award for Scientific Excellence in DiabetesAwarded by the North Texas American Diabetes Association (2014) Everson Award for distinguished alumnipresented by the Department of Biochemistry, University of Wisconsin-Madison (2002)
