Anti-SDHB Antibody (CAB1809)
- SKU:
- CAB1809
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Research Area:
- Cell Biology
Description
抗体名: | Anti-SDHB Rabbit Monoclonal Antibody |
抗体コード: | CAB1809 |
抗体サイズ: | 20uL, 50uL, 100uL |
申し込み: | WB |
反応性: | Human, Mouse, Rat |
宿主種: | Rabbit |
免疫原: | A synthesized peptide derived from human SDHB |
申し込み: | WB |
推奨希釈: | WB 1:500 - 1:2000 |
反応性: | Human, Mouse, Rat |
ポジティブサンプル: | HepG2, U-87MG, Mouse lung, Mouse liver, Mouse brain, Rat liver, Rat brain |
免疫原: | A synthesized peptide derived from human SDHB |
精製方法: | Affinity purification |
ストレージバッファ: | Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 0.05% BSA, 50% glycerol, pH7.3. |
アイソタイプ: | IgG |
順序: | Email for sequence |
遺伝子ID: | 6390 |
Uniprot: | P21912 |
セルラーロケーション: | |
計算された分子量: | 32kDa |
観察された分子量: | 32KDa |
同義語: | CWS2, IP, PGL4, SDH, SDH1, SDH2, SDHIP |
バックグラウンド: | 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 Protein Function: | 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. |
UniProt Protein Details: | 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 |
NCBI Summary: | 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 Code: | P21912 |
NCBI GenInfo Identifier: | 20455488 |
NCBI Gene ID: | 6390 |
NCBI Accession: | P21912.3 |
UniProt Related Accession: | P21912 |
Molecular Weight: | |
NCBI Full Name: | Succinate dehydrogenase |
NCBI 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 |
UniProt Synonym Protein Names: | Iron-sulfur subunit of complex II; Ip |
Protein Family: | Succinate dehydrogenase |
UniProt Gene Name: | SDHB |
UniProt Entry Name: | SDHB_HUMAN |