Tricarboxylic acid cycle enzyme activities in a mouse model of methylmalonic aciduria

• Main Authors: Parith Wongkittichote, Gary Cunningham, Marshall L. Summar, Elena Pumbo, Patrick Forny, Matthias R. Baumgartner, Kimberly A. Chapman
• Other Authors: St. Louis Children's Hospital
• Format: Article
• Published: 2020
• Subjects: Biochemistry, Genetics and Molecular Biology; Medicine
• Online Access: https://repository.li.mahidol.ac.th/handle/123456789/50017
• Institution: Mahidol University

© 2019 The Authors Methylmalonic acidemia (MMA) is a propionate pathway disorder caused by dysfunction of the mitochondrial enzyme methylmalonyl-CoA mutase (MMUT). MMUT catalyzes the conversion of methylmalonyl-CoA to succinyl-CoA, an anaplerotic reaction which feeds into the tricarboxylic acid (TCA...