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[KO验证抗体] Rabbit Anti-SDHB  antibody (bsm-54180R )
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说明书: 50ul  100ul  
50ul/1580.00元
100ul/2500.00元
大包装/询价
产品编号 bsm-54180R
英文名称 [KO验证抗体] SDHB
中文名称 铬细胞瘤患者抑癌基因SDHB重组兔单抗
别    名 succinate dehydrogenase complex,subunit B; mitochondrial; DHSB_HUMAN; Ip; Iron sulfur subunit; Iron sulfur subunit of complex II; Iron-sulfur subunit of complex II; PGL 4; PGL4; SDH 1; SDH; SDH1; SDH2; SdhB; SDHIP; Succinate dehydrogenase [ubiquinone] iron sulfur protein mitochondrial; Succinate dehydrogenase [ubiquinone] iron-sulfur subunit; Succinate Dehydrogenase 1 Iron Sulfur Subunit; Succinate dehydrogenase complex subunit B iron sulfur; Succinate Dehydrogenase Complex Subunit B Iron Sulfur Protein; Succinate dehydrogenase iron sulfur protein.  
研究领域 肿瘤  细胞生物  信号转导  
抗体来源 Rabbit
克隆类型 Recombinant
交叉反应 (predicted: Human,Mouse,Rat)
产品应用 WB=1:500-2000, IHC-P=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 32kDa
细胞定位 细胞浆 细胞膜 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 Recombinant human SDHB 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 ron-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).

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).

Subunit:
Component of complex II composed of four subunits: the flavoprotein (FP) SDHA, iron-sulfur protein (IP) SDHB, and a cytochrome b560 composed of SDHC and SDHD.

Subcellular Location:
Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.

DISEASE:
Defects in SDHB are a cause of susceptibility to pheochromocytoma (PCC) [MIM:171300]. 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) [MIM:115310]. 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) [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 incomplete penetrance.

Similarity:
Belongs to the succinate dehydrogenase/fumarate reductase iron-sulfur protein family.
Contains 1 2Fe-2S ferredoxin-type domain.
Contains 1 4Fe-4S ferredoxin-type domain.

SWISS:
P21912

Gene ID:
6390

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