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"Characterizing the molecular architecture of the ...
Pharmacology & Chemical Biology Seminar Series
4/8/2021 - 12:00 PM-1:00 PM
"Characterizing the molecular architecture of the mitochondrial matrix: implications for treatment of inborn errors of metabolism"
Jerry Vockley, M.D., Ph.D.
Division Director, Genetic and Genomic Medicine
Cleveland Family Endowed Chair in Pediatric Research
University of Pittsburgh School of Medicine
Mitochondrial energy metabolism involves the interactions of three metabolic pathways: fatty acid oxidation, the tricarboxylic acid cycle, and oxidative phosphorylation. We have characterized the functional interactions of these pathways, mapped their physical interactions, and demonstrated the secondary effects of primary inborn errors of long chain fatty acid oxidation. these disorders are among the most common inborn errors of metabolism identified by newborn screening, but still suffer from significant morbidity and mortality. A better understanding of their pathophysiology has allowed development of new therapies for patients.
Contact
Melanie Hoffner
412/383-7757