| Gene Symbol |
SDHB
|
| Gene Synonyms |
0710008N11Rik CWS2 IP MC2DN4 PGL4 PPGL4 SDH SDH1 SDH2 SDHIP |
| Gene Full Name |
succinate dehydrogenase complex iron sulfur subunit B |
| Gene Summary |
This tumor suppressor gene encodes the iron-sulfur protein subunit of the succinate dehydrogenase (SDH) enzyme complex which plays a critical role in mitochondria. The SDH enzyme complex is composed of four nuclear-encoded subunits. This enzyme complex converts succinate to fumarate which releases electrons as part of the citric acid cycle, and the enzyme complex additionally provides an attachment site for released electrons to be transferred to the oxidative phosphorylation pathway. The SDH enzyme complex plays a role in oxygen-related gene regulation through its conversion of succinate, which is an oxygen sensor that stabilizes the hypoxia-inducible factor 1 (HIF1) transcription factor. Sporadic and familial mutations in this gene result in paragangliomas, pheochromocytoma, and gastrointestinal stromal tumors, supporting a link between mitochondrial dysfunction and tumorigenesis. Mutations in this gene are also implicated in nuclear type 4 mitochondrial complex II deficiency. [provided by RefSeq, Jun 2022] |
| Alternative Names |
FLJ92337 IP iron-sulfur subunit iron-sulfur subunit of complex II malate dehydrogenase [quinone] iron-sulfur subunit OTTHUMP00000044624 succinate dehydrogenase complex subunit B succinate dehydrogenase complex subunit B iron sulfur (Ip) succinate-ubiquinone oxidoreductase Ip subunit precursor |
| Molecular Weight(MW) |
31kDa |
| Source |
Rabbit |
| Cellular Localization |
Mitochondrion inner membrane;Peripheral membrane protein |