Recombinant Human Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial (SDHB)

Uniprot Id

P21912

Target Species

Human

Target Name

SDHB

Target Full Name

Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial

Target 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).

Target Involvement

Pheochromocytoma (PCC); Paragangliomas 4 (PGL4); Paraganglioma and gastric stromal sarcoma (PGGSS); Cowden syndrome 2 (CWS2)

Target Subcellular Location

Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.

Target Protein Families

Succinate dehydrogenase/fumarate reductase iron-sulfur protein family

Target Synonyms

CWS2; DHSB_HUMAN; FLJ92337; Ip; Iron sulfur subunit; Iron sulfur subunit of complex II; Iron-sulfur subunit of complex II; mitochondrial; PGL 4; PGL4; SDH 1; SDH; SDH1; SDH2; SDH2, homolog of; SdhB; SDHIP; Succinate dehydrogenase [ubiquinone] iron sulfur protein mitochondrial; Succinate dehydrogenase [ubiquinone] iron sulfur subunit; Succinate dehydrogenase [ubiquinone] iron-sulfur subunit; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial; Succinate Dehydrogenase 1 Iron Sulfur Subunit; Succinate Dehydrogenase 2, S. cerevisiae, homolog of; Succinate dehydrogenase complex iron sulfur subunit B; Succinate dehydrogenase complex subunit B iron sulfur; Succinate Dehydrogenase Complex Subunit B Iron Sulfur Protein; succinate dehydrogenase complex, subunit B, iron sulfur (Ip); Succinate dehydrogenase iron sulfur protein

Target Background

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.