Mitochondrial genome linearization is a causative factor for cardiomyopathy in mice and Drosophila.

ABSTRACT:

Aims

Mitofusin (Mfn)2 redundantly promotes mitochondrial outer membrane tethering and organelle fusion with Mfn1, and uniquely functions as the mitochondrial receptor for Parkin during PTEN-induced putative kinase 1 (PINK1)-Parkin-mediated mitophagy. Selective deletion of Mfn2 with retention of Mfn1 preserves mitochondrial fusion while rendering damaged mitochondria resistant to normal quality control culling mechanisms. Consequently, neuron and cardiomyocyte-specific Mfn2 gene ablation is associated with accumulation of damaged mitochondria and organ dysfunction. Here, we determined how mitochondrial DNA (mtDNA) damage contributes to cardiomyopathy in Mfn2-deficient hearts.

Results

RNA sequencing of Mfn2-deficient hearts revealed increased expression of some nuclear-encoded

SUBMITTER: Chen Y 

PROVIDER: S-EPMC4208594 | biostudies-literature | 2014 Nov

REPOSITORIES: biostudies-literature