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 Polyclonal Antibody detects endogenous levels of SDHB protein.
来源
Polyclonal, Rabbit,IgG
组成(Formulation)
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
稀释比例
WB: 1/500 - 1/2000. IHC-p: 1/100-1/300. ELISA: 1/20000.. IF 1:50-200
纯化工艺(Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
背景(Background)
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
蛋白名称
Succinate dehydrogenase [ubiquinone] iron-sulfur subunit mitochondrial
其他名称
SDHB; SDH; SDH1; Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial; Iron-sulfur subunit of complex II; Ip
Fields
>>Citrate cycle (TCA cycle);>>Oxidative phosphorylation;>>Metabolic pathways;>>Carbon metabolism;>>Thermogenesis;>>Non-alcoholic fatty liver disease;>>Alzheimer disease;>>Parkinson disease;>>Amyotrophic lateral sclerosis;>>Huntington disease;>>Prion disease;>>Pathways of neurodegeneration - multiple diseases;>>Chemical carcinogenesis - reactive oxygen species;>>Diabetic cardiomyopathy
细胞定位
Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.
组织表达
Brain,Fibroblast,Liver,