UniProt Summary: Plays an essential role in the assembly of succinate dehydrogenase (SDH), an enzyme complex (also referred to as respiratory complex II) that is a component of both the tricarboxylic acid (TCA) cycle and the mitochondrial electron transport chain, and which couples the oxidation of succinate to fumarate with the reduction of ubiquinone (coenzyme Q) to ubiquinol. Promotes maturation of the iron-sulfur protein subunit SDHB of the SDH catalytic dimer, protecting it from the deleterious effects of oxidants. May act together with SDHAF1. {ECO:0000250|UniProtKB:Q04401, ECO:0000250|UniProtKB:Q8SZ16}.
Pfam DomainsGO Terms
Pfam Domains
Complex1 LYR
GO Terms
mitochondrial respiratory chain complex II assembly
respiratory chain complex II assembly
succinate metabolic process
regulation of gluconeogenesis
mitochondrial intermembrane space
regulation of carbohydrate biosynthetic process
mitochondrial respiratory chain complex assembly
dicarboxylic acid metabolic process
regulation of glucose metabolic process
regulation of cellular carbohydrate metabolic process
regulation of carbohydrate metabolic process
mitochondrial matrix
regulation of small molecule metabolic process
mitochondrion organization
drug metabolic process
cellular protein-containing complex assembly
carboxylic acid metabolic process
oxoacid metabolic process
organic acid metabolic process
protein-containing complex assembly
small molecule metabolic process
protein-containing complex subunit organization
CRISPR Data
Compound HitMost Correlated Genes in ChemogenomicsTissues where Essential in the Avana Dataset (DepMap 20Q1)