Ontology highlight
ABSTRACT:
SUBMITTER: Sokol AM
PROVIDER: S-EPMC6245507 | biostudies-literature | 2018 Nov
REPOSITORIES: biostudies-literature
Sokol Anna M AM Uszczynska-Ratajczak Barbara B Collins Michelle M MM Bazala Michal M Topf Ulrike U Lundegaard Pia R PR Sugunan Sreedevi S Guenther Stefan S Kuenne Carsten C Graumann Johannes J Chan Sherine S L SSL Stainier Didier Y R DYR Chacinska Agnieszka A
PLoS genetics 20181120 11
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 ...[more]