Mitochondrial cardiomyopathies feature increased uptake and diminished efflux of mitochondrial calcium.

ABSTRACT: Calcium (Ca²⁺) influx into the mitochondrial matrix stimulates ATP synthesis. Here, we investigate whether mitochondrial Ca²⁺ transport pathways are altered in the setting of deficient mitochondrial energy synthesis, as increased matrix Ca²⁺ may provide a stimulatory boost. We focused on mitochondrial cardiomyopathies, which feature such dysfunction of oxidative phosphorylation. We study a mouse model where the main transcription factor for mitochondrial DNA (transcription factor A, mitochondrial, Tfam) has been disrupted selectively in cardiomyocytes. By the second postnatal week (10-15day old mice), these mice have developed a dilated cardiomyopathy associated with impaired oxidative phosphorylation. We find evidence of increased mitochondrial Ca^2+</

SUBMITTER: Sommakia S 

PROVIDER: S-EPMC5652072 | biostudies-literature | 2017 Dec

REPOSITORIES: biostudies-literature