succinate dehydrogenase (mitochondrial complex 2)

Function: carbohydrate metabolism; tricarboxylic acid cycle succinate + ubiquinone fumarate + ubiquinol Structure: - four subunits: - succinate dehydrogenase flavoprotein subunit (SDHA) - succinate dehydrogenase iron-sulfur subunit (SDHB) - cytochrome b560 (SDHC) - succinate dehydrogenase complex subunit D (SDHD) Compartment: mitochondria inner membrane Pathology: - defects in subunits associated with succinate CoQ reductase deficiency

Related

citric acid cycle, Krebs cycle or tricarboxylic acid [TCA] cycle electron transport succinate CoQ reductase deficiency (complex 2 mitochondrial respiratory chain deficiency)

General

citric acid cycle enzyme dehydrogenase electron transport enzyme multisubunit protein quinone reductase

Properties

SIZE: MW = 140 kD COMPARTMENT: inner mitochondrial membrane MOTIF: active site SUBUNITS: succinate dehydrogenase flavoprotein subunit MOTIF: cofactor-binding site [64-78] COFACTOR-BOUND: flavin adenine dinucleotide Thr phosphorylation site {T252} histidine residue {H296} arginine residue {R312} succinate dehydrogenase iron-sulfur subunit MOTIF: 2Fe-2S ferredoxin-type {40-133} MOTIF: cofactor-binding site [93-93] COFACTOR-BOUND: iron sulfur [FeS] cluster cofactor-binding site [98-98] COFACTOR-BOUND: iron sulfur [FeS] cluster cofactor-binding site [101-101] COFACTOR-BOUND: iron sulfur [FeS] cluster cofactor-binding site [113-113] COFACTOR-BOUND: iron sulfur [FeS] cluster cofactor-binding site [186-186] COFACTOR-BOUND: iron sulfur [FeS] cluster cofactor-binding site [189-189] COFACTOR-BOUND: iron sulfur [FeS] cluster cofactor-binding site [192-192] COFACTOR-BOUND: iron sulfur [FeS] cluster cofactor-binding site [196-196] COFACTOR-BOUND: iron sulfur [FeS] cluster cofactor-binding site [243-243] COFACTOR-BOUND: iron sulfur [FeS] cluster cofactor-binding site [249-249] COFACTOR-BOUND: iron sulfur [FeS] cluster cofactor-binding site [253-253] COFACTOR-BOUND: iron sulfur [FeS] cluster cytochrome b560 MOTIF: transmembrane domain {71-93} transmembrane domain {105-127} MOTIF: Iron [Fe]-binding site SITE: 127-127 transmembrane domain {147-168} succinate dehydrogenase complex subunit D MOTIF: transmembrane domain {71-91} Iron [Fe]-binding site SITE: 102-102 Iron [Fe]-binding site SITE: 104-104 transmembrane domain {126-142}

References

UniProt :accession P21912

Components

succinate dehydrogenase cytochrome b small subunit (cybS, succinate dehydrogenase complex subunit D, SDHD) succinate dehydrogenase cytochrome b560 subunit (succinate dehydrogenase complex subunit C, succinate-ubiquinone oxidoreductase cytochrome B large subunit, CYBL, SDHC, CYB560, SDH3) succinate dehydrogenase flavoprotein subunit (SDHA, SDH2, SDHF) succinate dehydrogenase iron-sulfur subunit (SDHB)