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 (PubMed:24954416). Binds to the flavoprotein subunit SDHA in its FAD-bound form, blocking the generation of excess reactive oxigen species (ROS) and facilitating its assembly with the iron-sulfur protein subunit SDHB into the SDH catalytic dimer (By similarity). {ECO:0000250|UniProtKB:P38345, ECO:0000269|PubMed:24954416}.
Pfam DomainsGO Terms
Pfam Domains
DUF1674
GO Terms
mitochondrial respiratory chain complex II assembly
respiratory chain complex II assembly
succinate dehydrogenase (ubiquinone) activity
mitochondrial respiratory chain complex II, succinate dehydrogenase complex (ubiquinone)
neural retina development
mitochondrial respiratory chain complex assembly
retina development in camera-type eye
cellular respiration
energy derivation by oxidation of organic compounds
camera-type eye development
eye development
visual system development
sensory system development
mitochondrial matrix
generation of precursor metabolites and energy
mitochondrion organization
cellular component
sensory organ development
molecular function
innate immune response
cellular protein-containing complex assembly
defense response to other organism
oxidation-reduction process
response to other organism
response to external biotic stimulus
response to biotic stimulus
defense response
protein-containing complex assembly
protein-containing complex subunit organization
immune response
CRISPR Data
Compound HitMost Correlated Genes in ChemogenomicsTissues where Essential in the Avana Dataset (DepMap 20Q1)