Loss of the Mia40a oxidoreductase leads to hepato-pancreatic insufficiency in zebrafish.

ABSTRACT: Development and function of tissues and organs are powered by the activity of mitochondria. In humans, inherited genetic mutations that lead to progressive mitochondrial pathology often manifest during infancy and can lead to death, reflecting the indispensable nature of mitochondrial biogenesis and function. Here, we describe a zebrafish mutant for the gene mia40a (chchd4a), the life-essential homologue of the evolutionarily conserved Mia40 oxidoreductase which drives the biogenesis of cysteine-rich mitochondrial proteins. We report that mia40a mutant animals undergo progressive cellular respiration defects and develop enlarged mitochondria in skeletal muscles before their ultimate death at the larval stage. We generated a deep transcriptomic and proteomic resource that allowed us to iden

SUBMITTER: Sokol AM 

PROVIDER: S-EPMC6245507 | biostudies-literature | 2018 Nov

REPOSITORIES: biostudies-literature