反应种属
Human;Mouse;Rat;Fish
蛋白名称
Succinate dehydrogenase [ubiquinone] iron-sulfur subunit mitochondrial
免疫原
The antiserum was produced against synthesized peptide derived from the Internal region of human SDHB. AA range:131-180
特异性
SDHB Monoclonal Antibody detects endogenous levels of SDHB protein.
组成
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
纯化工艺
The antibody was affinity-purified from mouse antiserum by affinity-chromatography using epitope-specific immunogen.
其他名称
SDHB; SDH; SDH1; Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial; Iron-sulfur subunit of complex II; Ip
背景
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],
功能
catalytic activity:Succinate + ubiquinone = fumarate + ubiquinol.,cofactor:Binds 1 2Fe-2S cluster.,cofactor:Binds 1 3Fe-4S cluster.,cofactor:Binds 1 4Fe-4S cluster.,disease:Defects in SDHB are a cause of Cowden-like syndrome [MIM:612359]. Cowden-like syndrome is a cancer predisposition syndrome associated with elevated risk for tumors of the breast, thyroid, kidney and uterus.,disease:Defects in SDHB are a cause of paraganglioma and gastric stromal sarcoma [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 inc
