Entrez Summary: Inner mitochondrial membrane complex III (CIII) is the main enzyme complex in the mitochondrial respiratory chain, and Rieske Fe-S protein (UQCRFS1) is the last catalytic subunit added to the complex. The protein encoded by this gene is a nuclear-encoded mitochondrial matrix protein that stabilizes UQCRFS1 and chaperones it to the CIII complex. Defects in this gene are a cause of mitochondrial complex III deficiency, nuclear type 8. Three transcript variants encoding two different isoforms have been found for this gene. [provided by RefSeq, Jun 2014].
UniProt Summary: Assembly factor required for Rieske Fe-S protein UQCRFS1 incorporation into the cytochrome b-c1 (CIII) complex. Functions as a chaperone, binding to this subunit within the mitochondrial matrix and stabilizing it prior to its translocation and insertion into the late CIII dimeric intermediate within the mitochondrial inner membrane. {ECO:0000269|PubMed:23168492}.
Pfam DomainsGO Terms
Pfam Domains
Complex1 LYR
GO Terms
respiratory chain complex III assembly
mitochondrial respiratory chain complex III assembly
cytochrome complex assembly
mitochondrial respiratory chain complex assembly
mitochondrial membrane
cellular respiration
energy derivation by oxidation of organic compounds
mitochondrial matrix
generation of precursor metabolites and energy
mitochondrion organization
cellular protein-containing complex assembly
oxidation-reduction process
protein-containing complex assembly
protein-containing complex subunit organization
CRISPR Data
Compound HitMost Correlated Genes in ChemogenomicsTissues where Essential in the Avana Dataset (DepMap 20Q1)