Entrez Summary: Complex II of the respiratory chain, which is specifically involved in the oxidation of succinate, carries electrons from FADH to CoQ. The complex is composed of four nuclear-encoded subunits and is localized in the mitochondrial inner membrane. The iron-sulfur subunit is highly conserved and contains three cysteine-rich clusters which may comprise the iron-sulfur centers of the enzyme. Sporadic and familial mutations in this gene result in paragangliomas and pheochromocytoma, and support a link between mitochondrial dysfunction and tumorigenesis. [provided by RefSeq, Jul 2008].
UniProt Summary: Iron-sulfur protein (IP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q).
Pfam DomainsGO Terms
Pfam Domains
No Pfam Domain information is available for this gene.
GO Terms
3 iron, 4 sulfur cluster binding
mitochondrial respiratory chain complex II, succinate dehydrogenase complex (ubiquinone)
succinate dehydrogenase (ubiquinone) activity
succinate metabolic process
ubiquinone binding
2 iron, 2 sulfur cluster binding
tricarboxylic acid cycle
4 iron, 4 sulfur cluster binding
electron transfer activity
aerobic respiration
dicarboxylic acid metabolic process
mitochondrial membrane
respiratory electron transport chain
cellular respiration
electron transport chain
energy derivation by oxidation of organic compounds
mitochondrial inner membrane
generation of precursor metabolites and energy
drug metabolic process
carboxylic acid metabolic process
oxidation-reduction process
oxoacid metabolic process
organic acid metabolic process
mitochondrion
small molecule metabolic process
CRISPR Data
Compound HitMost Correlated Genes in ChemogenomicsTissues where Essential in the Avana Dataset (DepMap 20Q1)