Tissue-specific characterization of mitochondrial branched-chain keto acid oxidation using a multiplexed assay platform.

ABSTRACT: Alterations to branched-chain keto acid (BCKA) oxidation have been implicated in a wide variety of human diseases, ranging from diabetes to cancer. Although global shifts in BCKA metabolism-evident by gene transcription, metabolite profiling, and in vivo flux analyses have been documented across various pathological conditions, the underlying biochemical mechanism(s) within the mitochondrion remain largely unknown. In vitro experiments using isolated mitochondria represent a powerful biochemical tool for elucidating the role of the mitochondrion in driving disease. Such analyses have routinely been utilized across disciplines to shed valuable insight into mitochondrial-linked pathologies. That said, few studies have attempted to model in vitro BCKA oxidation in isolate

SUBMITTER: Goldberg EJ 

PROVIDER: S-EPMC7071349 | biostudies-literature | 2019 May

REPOSITORIES: biostudies-literature