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[KO驗證抗體] Rabbit Anti-SDHB  antibody (bsm-54180R )  
~~~促銷代碼KT202411~~~
訂購熱線:400-901-9800
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說明書: 50ul  100ul  
50ul/1400.00元
100ul/2500.00元
大包裝/詢價
產品編號 bsm-54180R
英文名稱 [KO驗證抗體] Rabbit Anti-SDHB  antibody
中文名稱 鉻細胞瘤患者抑癌基因SDHB重組兔單抗
別    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.  
研究領域 腫瘤  細胞生物  信號轉導  
抗體來源 Rabbit
克隆類型 Recombinant
交叉反應 (predicted: Human,Mouse,Rat)
產品應用 WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:400-800,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 32kDa
細胞定位 細胞漿 細胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 Recombinant human SDHB 
亞    型 IgG
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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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