succinate dehydrogenase iron-sulfur subunit (SDHB)

Function: - nuclear-encoded iron-sulfur subunit of mitochondrial complex 2 - carbohydrate metabolism; tricarboxylic acid cycle succinate + ubiquinone fumarate + ubiquinol Cofactor: - binds 1 2Fe-2S cluster (putative) - binds 1 3Fe-4S cluster (putative) - binds 1 4Fe-4S cluster (putative) Structure: - belongs to the succinate dehydrogenase/fumarate reductase iron-sulfur protein family - contains 1 2Fe-2S ferredoxin-type domain - contains 1 4Fe-4S type ferredoxin domain Compartment: mitochondria inner membrane Pathology: - defects in SDHB are a cause of pheochromocytoma - defects in SDHB are the cause of hereditary paraganglioma 4

General

flavoprotein protein subunit

Properties

SIZE: entity length = 280 aa MW = 32 kD COMPARTMENT: mitochondria 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

Database Correlations

OMIM correlations MORBIDMAP 185470 UniProt P21912 Pfam PF00111 Kegg hsa:6390 ENZYME 1.3.5.1

References

UniProt :accession P21912