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Rabbit Anti-ECE1/Gold Conjugated antibody (bs-1190R-Gold)
~~~促销,代码KXJ230206-6~~~
订购热线:400-901-9800
订购邮箱:sales@bioss.com.cn
订购QQ:  400-901-9800
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说 明 书: 100ul(10nm  15nm  35nm
100ul/2980.00元
大包装/询价
产品编号 bs-1190R-Gold
英文名称 Anti-ECE1/Gold
中文名称 胶体金标记的内皮素转化酶1抗体
别    名 ECE 1; ECE-1; Endothelin converting enzyme 1; ECE1_HUMAN.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul(10nm  15nm  35nm
研究领域 肿瘤  免疫学  信号转导  激酶和磷酸酶  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Rat,  (predicted: Human, Mouse, Dog, Pig, Cow, Rabbit, Guinea Pig, )
产品应用 IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 85kDa
性    状 Lyophilized or Liquid
浓    度 0.4mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ECE1 C-terminus
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300.
保存条件 Store at 2-8 ºC for 3-6 months. Avoid repeated freeze/thaw cycles.
产品介绍 background:
The protein encoded by this gene is involved in proteolytic processing of endothelin precursors to biologically active peptides. Mutations in this gene are associated with Hirschsprung disease, cardiac defects and autonomic dysfunction. Alternatively spliced transcript variants encoding different isoforms have been noted for this gene.[provided by RefSeq, Sep 2009].

Function:
Converts big endothelin-1 to endothelin-1.

Subunit:
Homodimer; disulfide-linked.

Subcellular Location:
Cell Membrane. Single pass type II membrane protein.

Tissue Specificity:
All isoforms are expressed in umbilical vein endothelial cells, polynuclear neutrophils, fibroblasts, atrium cardiomyocytes and ventricles. Isoforms A, B and C are also expressed in placenta, lung, heart, adrenal gland and phaeochromocytoma; isoforms A and C in liver, testis and small intestine; isoform B, C and D in endothelial cells and umbilical vein smooth muscle cells; isoforms C and D in saphenous vein cells, and isoform C in kidney.

DISEASE:
Defects in ECE1 are a cause of Hirschsprung disease cardiac defects and autonomic dysfunction (HSCRCDAD) [MIM:613870]. It is a form of Hirschsprung disease with skip-lesions defects, craniofacial abnormalities and other dysmorphic features, and autonomic dysfunction.

Similarity:
Belongs to the peptidase M13 family.

Database links:

Entrez Gene: 281133 Cow

Entrez Gene: 1889 Human

Entrez Gene: 230857 Mouse

Entrez Gene: 94204 Rat

Omim: 600423 Human

SwissProt: P42891 Cow

SwissProt: P42892 Human

SwissProt: Q4PZA2 Mouse

SwissProt: P42893 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

内皮素转化酶(endothelin—converting enzyme-1)是内皮素ET生物合成的关键酶,在体内内皮素生物活性调节上起着极为重要的作用。
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