LMPD Database

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LMP000586

UniProt Annotations

Entry Information
Gene Namesuccinate dehydrogenase complex, subunit B, iron sulfur (Ip)
Protein EntrySDHB_HUMAN
UniProt IDP21912
SpeciesHuman
Comments
Comment typeDescription
Catalytic ActivitySuccinate + a quinone = fumarate + a quinol.
CofactorName=[2Fe-2S] cluster; Xref=ChEBI
CofactorName=[3Fe-4S] cluster; Xref=ChEBI
CofactorName=[4Fe-4S] cluster; Xref=ChEBI
DiseaseCowden syndrome 2 (CWS2) [MIM
DiseaseParaganglioma and gastric stromal sarcoma (PGGSS) [MIM
DiseaseParagangliomas 4 (PGL4) [MIM
DiseasePheochromocytoma (PCC) [MIM
FunctionIron-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).
InteractionP31040:SDHA; NbExp=2; IntAct=EBI-1056481, EBI-1057265;
PathwayCarbohydrate metabolism; tricarboxylic acid cycle; fumarate from succinate (eukaryal route): step 1/1.
SimilarityBelongs to the succinate dehydrogenase/fumarate reductase iron-sulfur protein family.
SimilarityContains 1 2Fe-2S ferredoxin-type domain.
SimilarityContains 1 4Fe-4S ferredoxin-type domain.
Subcellular LocationMitochondrion inner membrane; Peripheral membrane protein; Matrix side.
SubunitComponent of complex II composed of four subunits: the flavoprotein (FP) SDHA, iron-sulfur protein (IP) SDHB, and a cytochrome b560 composed of SDHC and SDHD.
Web ResourceName=Atlas of Genetics and Cytogenetics in Oncology and Haematology; URL="http://atlasgeneticsoncology.org/Genes/SDHBID388.html";
Web ResourceName=TCA Cycle Gene Mutation Database; URL="http://chromium.liacs.nl/LOVD2/SDH/home.php?select_db=SDHB";