Opa1 Overexpression Protects from Early-Onset Mpv17^-/--Related Mouse Kidney Disease.

ABSTRACT: Moderate overexpression of Opa1, the master regulator of mitochondrial cristae morphology, significantly improved mitochondrial damage induced by drugs, surgical denervation, or oxidative phosphorylation (OXPHOS) defects due to specific impairment of a single mitochondrial respiratory chain complex. Here, we investigated the effectiveness of this approach in the Mpv17^-/- mouse, characterized by profound, multisystem mitochondrial DNA (mtDNA) depletion. After the crossing with Opa1^tg mice, we found a surprising anticipation of the severe, progressive focal segmental glomerulosclerosis, previously described in Mpv17^-/- animals as a late-onset clinical feature (after 12-18 months of life). In contrast, Mpv17^-/- animals from this new "mixed" strain d

SUBMITTER: Luna-Sanchez M 

PROVIDER: S-EPMC7403474 | biostudies-literature | 2020 Aug

REPOSITORIES: biostudies-literature