Tafazzin deficiency impairs CoA-dependent oxidative metabolism in cardiac mitochondria
Catherine Le, Lindsay G. Benage, Kalyn S. Specht, Lance C. Li Puma, Christopher Mulligan, Adam L. Heuberger, Jessica E. Prenni, Steven M. Claypool, Kathryn C. Chatfield, Genevieve C. Sparagna, Adam J. Chicco
Abstract
mitochondria with exogenous CoA partially rescued pyruvate and palmitoylcarnitine oxidation capacities, implicating dysregulation of CoA-dependent intermediary metabolism rather than respiratory chain defects in the bioenergetic impacts of tafazzin deficiency. These findings support links among cardiolipin abnormalities, respiratory supercomplex instability, and mitochondrial oxidant production and shed new light on the distinct metabolic consequences of tafazzin deficiency in the mammalian heart.
Topics & Concepts
MitochondrionOxidative phosphorylationOxidative metabolismMetabolismEnergy metabolismChemistryBiochemistryCell biologyBiologyEndocrinologyMitochondrial Function and PathologyMetabolomics and Mass Spectrometry StudiesMetabolism and Genetic Disorders