Mitochondrial Cytochrome c Oxidase Defects Alter Cellular Homeostasis of Transition Metals.

ABSTRACT: The redox activity of cytochrome c oxidase (COX), the terminal oxidase of the mitochondrial respiratory chain (MRC), depends on the incorporation of iron and copper into its catalytic centers. Many mitochondrial proteins have specific roles for the synthesis and delivery of metal-containing cofactors during COX biogenesis. In addition, a large set of different factors possess other molecular functions as chaperones or translocators that are also necessary for the correct maturation of these complexes. Pathological variants in genes encoding structural MRC subunits and these different assembly factors produce respiratory chain deficiency and lead to mitochondrial disease. COX deficiency in Drosophila melanogaster, induced by downregulated expression of three different assembly

SUBMITTER: Brischigliaro M 

PROVIDER: S-EPMC9160823 | biostudies-literature | 2022

REPOSITORIES: biostudies-literature