ID:DHSB_HUMAN DESCRIPTION: RecName: Full=Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial; EC=1.3.5.1; AltName: Full=Iron-sulfur subunit of complex II; Short=Ip; Flags: Precursor; FUNCTION: Iron-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). CATALYTIC ACTIVITY: Succinate + ubiquinone = fumarate + ubiquinol. COFACTOR: Binds 1 2Fe-2S cluster (By similarity). COFACTOR: Binds 1 3Fe-4S cluster (By similarity). COFACTOR: Binds 1 4Fe-4S cluster (By similarity). PATHWAY: Carbohydrate metabolism; tricarboxylic acid cycle; fumarate from succinate (eukaryal route): step 1/1. SUBUNIT: Component 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. INTERACTION: P31040:SDHA; NbExp=2; IntAct=EBI-1056481, EBI-1057265; SUBCELLULAR LOCATION: Mitochondrion inner membrane; Peripheral membrane protein; Matrix side. DISEASE: Defects in SDHB are a cause of susceptibility to pheochromocytoma (PCC) [MIM:171300]. A catecholamine-producing tumor of chromaffin tissue of the adrenal medulla or sympathetic paraganglia. The cardinal symptom, reflecting the increased secretion of epinephrine and norepinephrine, is hypertension, which may be persistent or intermittent. DISEASE: Defects in SDHB are the cause of paragangliomas type 4 (PGL4) [MIM:115310]. A neural crest tumor usually derived from the chromoreceptor tissue of a paraganglion. Paragangliomas are most commonly located in the head and neck region, specifically at the carotid bifurcation, the jugular foramen, the vagal nerve, and in the middle ear. DISEASE: Defects in SDHB are a cause of paraganglioma and gastric stromal sarcoma (PGGSS) [MIM:606864]; also called Carney-Stratakis syndrome. Gastrointestinal stromal tumors may be sporadic or inherited in an autosomal dominant manner, alone or as a component of a syndrome associated with other tumors, such as in the context of neurofibromatosis type 1 (NF1). Patients have both gastrointestinal stromal tumors and paragangliomas. Susceptibility to the tumors was inherited in an apparently autosomal dominant manner, with incomplete penetrance. DISEASE: Defects in SDHB are a cause of Cowden-like syndrome (CWDLS) [MIM:612359]. Cowden-like syndrome is a cancer predisposition syndrome associated with elevated risk for tumors of the breast, thyroid, kidney and uterus. SIMILARITY: Belongs to the succinate dehydrogenase/fumarate reductase iron-sulfur protein family. SIMILARITY: Contains 1 2Fe-2S ferredoxin-type domain. SIMILARITY: Contains 1 4Fe-4S ferredoxin-type domain. WEB RESOURCE: Name=Atlas of Genetics and Cytogenetics in Oncology and Haematology; URL="http://atlasgeneticsoncology.org/Genes/SDHBID388.html"; WEB RESOURCE: Name=GeneReviews; URL="http://www.ncbi.nlm.nih.gov/sites/GeneTests/lab/gene/SDHB"; WEB RESOURCE: Name=TCA Cycle Gene Mutation Database; URL="http://chromium.liacs.nl/LOVD2/SDH/home.php?select_db=SDHB";
The RNAfold program from the Vienna RNA Package is used to perform the secondary structure predictions and folding calculations. The estimated folding energy is in kcal/mol. The more negative the energy, the more secondary structure the RNA is likely to have.
ModBase Predicted Comparative 3D Structure on P21912
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Orthologous Genes in Other Species
Orthologies between human, mouse, and rat are computed by taking the best BLASTP hit, and filtering out non-syntenic hits. For more distant species reciprocal-best BLASTP hits are used. Note that the absence of an ortholog in the table below may reflect incomplete annotations in the other species rather than a true absence of the orthologous gene.
LF383925 - JP 2014500723-A/191428: Polycomb-Associated Non-Coding RNAs. U17248 - Human succinate dehydrogenase iron-protein subunit (sdhB) gene, complete cds. D10245 - Homo sapiens sdh B mRNA for succinate-ubiquinone oxidoreductase iron sulfur subunit, partial cds. M32246 - Human succinate-ubiquinone oxidoreductase iron sulfur (Ip) subunit mRNA, 3' end. BC007840 - Homo sapiens succinate dehydrogenase complex, subunit B, iron sulfur (Ip), mRNA (cDNA clone MGC:14116 IMAGE:4124737), complete cds. JD262317 - Sequence 243341 from Patent EP1572962. JD288240 - Sequence 269264 from Patent EP1572962. JD062126 - Sequence 43150 from Patent EP1572962. JD082782 - Sequence 63806 from Patent EP1572962. AK312056 - Homo sapiens cDNA, FLJ92337, highly similar to Homo sapiens succinate dehydrogenase complex, subunit B, iron sulfur (Ip) (SDHB), mRNA. KJ897533 - Synthetic construct Homo sapiens clone ccsbBroadEn_06927 SDHB gene, encodes complete protein. KJ897534 - Synthetic construct Homo sapiens clone ccsbBroadEn_06928 SDHB gene, encodes complete protein. KR710093 - Synthetic construct Homo sapiens clone CCSBHm_00009505 SDHB (SDHB) mRNA, encodes complete protein. KR710094 - Synthetic construct Homo sapiens clone CCSBHm_00009510 SDHB (SDHB) mRNA, encodes complete protein. KR710095 - Synthetic construct Homo sapiens clone CCSBHm_00009530 SDHB (SDHB) mRNA, encodes complete protein. KR710096 - Synthetic construct Homo sapiens clone CCSBHm_00009545 SDHB (SDHB) mRNA, encodes complete protein. DQ403007 - Homo sapiens succinate dehydrogenase complex subunit B (SDHB) mRNA, partial cds. JD021864 - Sequence 2888 from Patent EP1572962. JD036009 - Sequence 17033 from Patent EP1572962. JD021379 - Sequence 2403 from Patent EP1572962. JD033509 - Sequence 14533 from Patent EP1572962. LF357199 - JP 2014500723-A/164702: Polycomb-Associated Non-Coding RNAs. JD218952 - Sequence 199976 from Patent EP1572962. MA619502 - JP 2018138019-A/191428: Polycomb-Associated Non-Coding RNAs. MA592776 - JP 2018138019-A/164702: Polycomb-Associated Non-Coding RNAs.
Biochemical and Signaling Pathways
BioCyc Knowledge Library PWY-3781 - aerobic respiration I (cytochrome c) PWY66-398 - TCA cycle PWY66-407 - superpathway of conversion of glucose to acetyl CoA and entry into the TCA cycle
BioCarta from NCI Cancer Genome Anatomy Project h_etcPathway - Electron Transport Reaction in Mitochondria
Reactome (by CSHL, EBI, and GO)
Protein P21912 (Reactome details) participates in the following event(s):
R-HSA-163213 Transfer of electrons through the succinate dehydrogenase complex R-HSA-70994 Succinate <=> Fumarate (with FAD redox reaction on enzyme) R-HSA-611105 Respiratory electron transport R-HSA-163200 Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins. R-HSA-71403 Citric acid cycle (TCA cycle) R-HSA-1428517 The citric acid (TCA) cycle and respiratory electron transport R-HSA-71406 Pyruvate metabolism and Citric Acid (TCA) cycle R-HSA-1430728 Metabolism
US Server
Sequence and Links to Tools and Databases 
Common Gene Haplotype Alleles 
