Bioenergetic consequences of compromised mitochondrial DNA repair in the mouse heart.

ABSTRACT: The progeroid phenotype of mitochondrial DNA (mtDNA) mutator mice has been nebulously attributed to general mitochondrial 'dysfunction', though few studies have rigorously defined the bioenergetic consequences of accumulating mtDNA mutations. Comprehensive mitochondrial diagnostics was employed to interrogate the bioenergetic properties of isolated cardiac mitochondria from mtDNA mutator mice and wild type littermates. Assessment of respiratory flux in conjunction with parallel measurements of mitochondrial free energy all point to the cause of respiratory flux limitations observed in mtDNA mutator mouse mitochondria being due to impairments within the energy transduction step catalyzed by the electron transport system in which NADH/NAD⁺ free energy is transduced to the proton m

SUBMITTER: McLaughlin KL 

PROVIDER: S-EPMC6173612 | biostudies-literature | 2018 Oct

REPOSITORIES: biostudies-literature