Rabbit SDHB Polyclonal Antibody | anti-SDHB antibody

SDHB Polyclonal Antibody

Cat. Num. AAA3011425

• Gene Names: SDHB; IP; SDH; CWS2; PGL4; SDH1; SDH2; SDHIP
• Reactivity: Human, Mouse, Rat
• Applications: Western Blot, Immunohistochemistry
• Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
• Synonyms: SDHB; Polyclonal Antibody; SDHB Polyclonal Antibody; Succinate dehydrogenase [ubiquinone] iron-sulfur subunit; mitochondrial; Iron-sulfur subunit of complex II; Ip; SDH; SDH1; anti-SDHB antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: SDHB polyclonal antibody detects endogenous levels of SDHB protein.
• Purity/Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
• Form/Format: Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
• Sequence Length: 280

• Applicable Applications for anti-SDHB antibody: Western Blot (WB), Immunohistochemistry (IHC)
• Application Notes: WB: 1:500-1:2000; IHC: 1:50-1:100
• Immunogen: Recombinant full length Human SDHB.
• Preparation and Storage: Store at 4 degree C short term.; Aliquot and store at -20 degree C long term.; Avoid freeze-thaw cycles.

Western Blot (WB)

(Western Blot (WB) analysis of SDHB polyclonal antibody)

Related Product Information for anti-SDHB antibody

In aerobic respiration reactions, succinate dehydrogenase (SDH) catalyzes the oxidation of succinate and ubiquinone to fumarate and ubiquinol. Four subunits comprise the SDH protein complex: a flavochrome subunit (SDHA), an iron-sulfur protein (SDHB) and two membrane-bound subunits (SDHC and SDHD) anchored to the inner mitochondrial membrane. Mutations to these subunits cause mitochondrial dysfunction, corresponding to several distinct disorders. Mutations in the membrane bound components may cause hereditary paraganglioma, while SDHA mutations associate with juvenile encephalopathy as well as Leigh syndrome, a severe neurological disorder. Inactivating mutations in SDHB correlate with inherited, but not necessarily sporadic, cases of pheochromocytoma.

NCBI and Uniprot Product Information

• NCBI GI #: 20455488
• NCBI GeneID: 6390
• UniProt Accession #: P21912
• Molecular Weight: ~ 32kDa
• NCBI Official Full Name: Succinate dehydrogenase
• NCBI Official Synonym Full Names: succinate dehydrogenase complex iron sulfur subunit B
• NCBI Official Symbol: SDHB
• NCBI Official Synonym Symbols: IP; SDH; CWS2; PGL4; SDH1; SDH2; SDHIP
• NCBI Protein Information: succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial
• UniProt Protein Name: Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial
• Protein Family: Succinate dehydrogenase
• UniProt Gene Name: SDHB
• UniProt Synonym Gene Names: SDH; SDH1; Ip
• UniProt Entry Name: SDHB_HUMAN

NCBI Description

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]

Uniprot Description

SDHB: 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). Defects in SDHB are a cause of susceptibility to pheochromocytoma (PCC). A catecholamine-producing tumor of chromaffin tissue of the adrenal medulla or sympathetic paraganglia. The cardinal symptom, reflecting the increased secretion of epinephrine and norepinephrine, is hypertension, which may be persistent or intermittent. Defects in SDHB are the cause of paragangliomas type 4 (PGL4). A neural crest tumor usually derived from the chromoreceptor tissue of a paraganglion. Paragangliomas are most commonly located in the head and neck region, specifically at the carotid bifurcation, the jugular foramen, the vagal nerve, and in the middle ear. Defects in SDHB are a cause of paraganglioma and gastric stromal sarcoma (PGGSS); 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 incomplete penetrance. Defects in SDHB are a cause of Cowden-like syndrome (CWDLS). Cowden-like syndrome is a cancer predisposition syndrome associated with elevated risk for tumors of the breast, thyroid, kidney and uterus. Belongs to the succinate dehydrogenase/fumarate reductase iron-sulfur protein family.

Protein type: EC 1.3.5.1; Mitochondrial; Carbohydrate Metabolism - citrate (TCA) cycle; Oxidoreductase; Energy Metabolism - oxidative phosphorylation

Chromosomal Location of Human Ortholog: 1p36.1-p35

Cellular Component: mitochondrial respiratory chain complex II; mitochondrion; mitochondrial inner membrane

Molecular Function: 2 iron, 2 sulfur cluster binding; ubiquinone binding; succinate dehydrogenase (ubiquinone) activity; protein binding; electron carrier activity; metal ion binding; 4 iron, 4 sulfur cluster binding; 3 iron, 4 sulfur cluster binding

Biological Process: succinate metabolic process; cellular metabolic process; tricarboxylic acid cycle; aerobic respiration

Disease: Gastrointestinal Stromal Tumor; Cowden Syndrome 2; Paraganglioma And Gastric Stromal Sarcoma; Paragangliomas 4; Pheochromocytoma