Gene/Proteome Database (LMPD)
LMPD ID
LMP000586
Gene ID
Species
Homo sapiens (Human)
Gene Name
succinate dehydrogenase complex, subunit B, iron sulfur (Ip)
Gene Symbol
Synonyms
CWS2; IP; PGL4; SDH; SDH1; SDH2; SDHIP
Alternate Names
succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial; iron-sulfur subunit of complex II
Chromosome
1
Map Location
1p36.1-p35
EC Number
1.3.5.1
Summary
Complex II of the respiratory chain, which is specifically involved in the oxidation of succinate, carries electrons from FADH to CoQ. The complex is composed of four nuclear-encoded subunits and is localized in the mitochondrial inner membrane. The iron-sulfur subunit is highly conserved and contains three cysteine-rich clusters which may comprise the iron-sulfur centers of the enzyme. Sporadic and familial mutations in this gene result in paragangliomas and pheochromocytoma, and support a link between mitochondrial dysfunction and tumorigenesis. [provided by RefSeq, Jul 2008]
Orthologs
Proteins
| succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial precursor | |
|---|---|
| Refseq ID | NP_002991 |
| Protein GI | 115387094 |
| UniProt ID | P21912 |
| mRNA ID | NM_003000 |
| Length | 280 |
| RefSeq Status | REVIEWED |
| MAAVVALSLRRRLPATTLGGACLQASRGAQTAAATAPRIKKFAIYRWDPDKAGDKPHMQTYEVDLNKCGPMVLDALIKIKNEVDSTLTFRRSCREGICGSCAMNINGGNTLACTRRIDTNLNKVSKIYPLPHMYVIKDLVPDLSNFYAQYKSIEPYLKKKDESQEGKQQYLQSIEEREKLDGLYECILCACCSTSCPSYWWNGDKYLGPAVLMQAYRWMIDSRDDFTEERLAKLQDPFSLYRCHTIMNCTRTCPKGLNPGKAIAEIKKMMATYKEKKASV | |
| transit_peptide: 1..28 calculated_mol_wt: 2840 peptide sequence: MAAVVALSLRRRLPATTLGGACLQASRG mat_peptide: 29..280 product: succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial calculated_mol_wt: 28808 peptide sequence: AQTAAATAPRIKKFAIYRWDPDKAGDKPHMQTYEVDLNKCGPMVLDALIKIKNEVDSTLTFRRSCREGICGSCAMNINGGNTLACTRRIDTNLNKVSKIYPLPHMYVIKDLVPDLSNFYAQYKSIEPYLKKKDESQEGKQQYLQSIEEREKLDGLYECILCACCSTSCPSYWWNGDKYLGPAVLMQAYRWMIDSRDDFTEERLAKLQDPFSLYRCHTIMNCTRTCPKGLNPGKAIAEIKKMMATYKEKKASV | |
Gene Information
Entrez Gene ID
Gene Name
succinate dehydrogenase complex, subunit B, iron sulfur (Ip)
Gene Symbol
Species
Homo sapiens
Gene Ontology (GO Annotations)
| GO ID | Source | Type | Description |
|---|---|---|---|
| GO:0070062 | IDA:UniProt | C | extracellular vesicular exosome |
| GO:0005743 | ISS:UniProtKB | C | mitochondrial inner membrane |
| GO:0005749 | ISS:UniProtKB | C | mitochondrial respiratory chain complex II |
| GO:0005739 | TAS:ProtInc | C | mitochondrion |
| GO:0051537 | ISS:UniProtKB | F | 2 iron, 2 sulfur cluster binding |
| GO:0051538 | ISS:UniProtKB | F | 3 iron, 4 sulfur cluster binding |
| GO:0051539 | ISS:UniProtKB | F | 4 iron, 4 sulfur cluster binding |
| GO:0009055 | IEA:InterPro | F | electron carrier activity |
| GO:0046872 | IEA:UniProtKB-KW | F | metal ion binding |
| GO:0008177 | IEA:UniProtKB-EC | F | succinate dehydrogenase (ubiquinone) activity |
| GO:0048039 | ISS:UniProtKB | F | ubiquinone binding |
| GO:0009060 | TAS:ProtInc | P | aerobic respiration |
| GO:0044237 | TAS:Reactome | P | cellular metabolic process |
| GO:0022904 | TAS:Reactome | P | respiratory electron transport chain |
| GO:0044281 | TAS:Reactome | P | small molecule metabolic process |
| GO:0006105 | IEA:Ensembl | P | succinate metabolic process |
| GO:0006099 | TAS:Reactome | P | tricarboxylic acid cycle |
KEGG Pathway Links
Domain Information
InterPro Annotations
| Accession | Description |
|---|---|
| IPR006058 | 2Fe-2S ferredoxin, iron-sulphur binding site |
| IPR001041 | 2Fe-2S ferredoxin-type domain |
| IPR017900 | 4Fe-4S ferredoxin, iron-sulphur binding, conserved site |
| IPR017896 | 4Fe-4S ferredoxin-type, iron-sulphur binding domain |
| IPR009051 | Alpha-helical ferredoxin |
| IPR012675 | Beta-grasp domain |
| IPR025192 | Succinate dehydogenase/fumarate reductase N-terminal |
| IPR004489 | Succinate dehydrogenase/fumarate reductase iron-sulphur protein |
UniProt Annotations
Entry Information
Gene Name
succinate dehydrogenase complex, subunit B, iron sulfur (Ip)
Protein Entry
SDHB_HUMAN
UniProt ID
Species
Human
Comments
| Comment Type | Description |
|---|---|
| Catalytic Activity | Succinate + a quinone = fumarate + a quinol. |
| Cofactor | Name=[2Fe-2S] cluster; Xref=ChEBI |
| Cofactor | Name=[3Fe-4S] cluster; Xref=ChEBI |
| Cofactor | Name=[4Fe-4S] cluster; Xref=ChEBI |
| Disease | Cowden syndrome 2 (CWS2) [MIM |
| Disease | Paraganglioma and gastric stromal sarcoma (PGGSS) [MIM |
| Disease | Paragangliomas 4 (PGL4) [MIM |
| Disease | Pheochromocytoma (PCC) [MIM |
| 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). |
| Interaction | P31040:SDHA; NbExp=2; IntAct=EBI-1056481, EBI-1057265; |
| Pathway | Carbohydrate metabolism; tricarboxylic acid cycle; fumarate from succinate (eukaryal route): step 1/1. |
| 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. |
| Subcellular Location | Mitochondrion inner membrane; Peripheral membrane protein; Matrix side. |
| 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. |
| Web Resource | Name=Atlas of Genetics and Cytogenetics in Oncology and Haematology; URL="http://atlasgeneticsoncology.org/Genes/SDHBID388.html"; |
| Web Resource | Name=TCA Cycle Gene Mutation Database; URL="http://chromium.liacs.nl/LOVD2/SDH/home.php?select_db=SDHB"; |
Identical and Related Proteins
Unique RefSeq proteins for LMP000586 (as displayed in Record Overview)
| Protein GI | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| 115387094 | RefSeq | NP_002991 | 280 | succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial precursor |
Identical Sequences to LMP000586 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:115387094 | GenBank | JAA16674.1 | 280 | succinate dehydrogenase complex, subunit B, iron sulfur (Ip) [Pan troglodytes] |
| GI:115387094 | GenBank | JAA22638.1 | 280 | succinate dehydrogenase complex, subunit B, iron sulfur (Ip) [Pan troglodytes] |
| GI:115387094 | GenBank | JAA37699.1 | 280 | succinate dehydrogenase complex, subunit B, iron sulfur (Ip) [Pan troglodytes] |
| GI:115387094 | GenBank | AIC55101.1 | 280 | SDHB, partial [synthetic construct] |
| GI:115387094 | GenBank | AIC55102.1 | 280 | SDHB, partial [synthetic construct] |
| GI:115387094 | RefSeq | XP_004024816.1 | 280 | PREDICTED: succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [Gorilla gorilla gorilla] |
Related Sequences to LMP000586 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:115387094 | GenBank | AAA81167.1 | 280 | succinate dehydrogenase iron-protein subunit [Homo sapiens] |
| GI:115387094 | GenBank | AED39209.1 | 280 | Sequence 1227 from patent US 7883858 |
| GI:115387094 | GenBank | AFL28757.1 | 280 | Sequence 752 from patent US 8168602 |
| GI:115387094 | GenBank | AHD76395.1 | 280 | Sequence 19668 from patent US 8586006 |
| GI:115387094 | RefSeq | XP_002811455.1 | 280 | PREDICTED: succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [Pongo abelii] |
| GI:115387094 | RefSeq | XP_007978514.1 | 280 | PREDICTED: succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [Chlorocebus sabaeus] |