Products of MIA40:ERV1 Substrates of MIA40:ERV1 MIA40:ERV1 (CHCHD4:GFER) Catalyzes Formation of Disulfide Bonds As inferred from the yeast MIA40:ERV1 complex, human CHCHD4 (MIA40 homolog) catalyzes the oxidation of cysteine residues in precursor proteins in the intermembrane space to form cystine bonds. The electrons from the cysteine residues are transferred to CHCHD4, then to GFER (ERV1 homolog), and eventually to the respiratory chain. The interaction between yeast MIA40 and ERV1 is transitory.<br>In yeast, experimentally verified substrates of MIA40:ERV1 include COX17, COX19, CMC2, CMC3, CMC4, TIM13 (TIMM13 in human), TIM9 (TIMM9 in human), TIM10 (TIMM10 in human), CCS1 (CCS in human), TIM8 (TIMM8 in human), and ERV1 (GFER in human). Many other mitochondrial proteins are anticipated to be substrates of the MIA40:ERV1 complex. Authored: May, B, 2011-05-23 Edited: May, B, 2011-05-23 Pubmed15683237 Pubmed16185709 Pubmed18174896 Pubmed19409522 Pubmed19453276 Pubmed20593814 Reactome Database ID Release 431307802 Reactome, http://www.reactome.org ReactomeREACT_118849 Reviewed: D'Eustachio, P, 2011-11-01 Reviewed: Endo, T, 2012-02-12