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货号： bs-5897R-AF350 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-5897R-AF350
- 英文名称
- Anti-Endothelin 3/AF350
- 中文名称
- AF350标记的内皮素3抗体
- 别 名
- ET 3; ET3; ET-3; PPET3; Preproendothelin 3; Preproendothelin3; Preproendothelin-3; Endothelin3; Endothelin-3; Endothelin 3; EDN3_HUMAN.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 肿瘤 免疫学 神经生物学 信号转导
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Dog, Pig, Cow, Rabbit,
- 产品应用
- IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 9/26kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human ET-3
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein A
- 储 存 液
- 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
- 保存条件
- Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
- 产品介绍
- background:
Endothelins are vasoactive proteins derived form the endothelium The active form of this protein is a 21aa peptide processed from the precursor. The active peptide binds endothelin receptor type B (EDNRB). This interaction EDNRB regulates the development of neural crest-derived cell lineages, such as melanocytes and enteric neurons. Mutations in Endothelin 3 and EDNRB have been associated with Hirschsprung disease (HSCR) and Waardenburg syndrome (WS), which are congenital disorders involving neural crest-derived cells.
Function:
Endothelins are endothelium-derived vasoconstrictor peptides.
Subcellular Location:
Secreted.
Tissue Specificity:
Expressed in trophoblasts and placental stem villi vessels, but not in cultured placental smooth muscle cells.
DISEASE:
Defects in EDN3 are the cause of Hirschsprung disease type 4 (HSCR4) [MIM:613712]; also known as aganglionic megacolon (MGC). A genetic disorder of neural crest development characterized by the absence of intramural ganglion cells in the hindgut; often resulting in intestinal obstruction.
Defects in EDN3 are a cause of congenital central hypoventilation syndrome (CCHS) [MIM:209880]; also known as congenital failure of autonomic control or Ondine curse. CCHS is a rare disorder characterized by abnormal control of respiration in the absence of neuromuscular or lung disease, or an identifiable brain stem lesion. A deficiency in autonomic control of respiration results in inadequate or negligible ventilatory and arousal responses to hypercapnia and hypoxemia.
Defects in EDN3 are a cause of Waardenburg syndrome type 4 (WS4B) [MIM:613265]; also known as Waardenburg-Shah syndrome. WS4B is characterized by the association of Waardenburg features (depigmentation and deafness) and the absence of enteric ganglia in the distal part of the intestine (Hirschsprung disease).
Similarity:
Belongs to the endothelin/sarafotoxin family.
Database links:Entrez Gene: 403406 Dog
Entrez Gene: 1908 Human
Entrez Gene: 13616 Mouse
Entrez Gene: 366270 Rat
Omim: 131242 Human
SwissProt: Q765Z4 Dog
SwissProt: P14138 Human
SwissProt: P48299 Mouse
SwissProt: P13207 Rat
Unigene: 1408 Human
Unigene: 9478 Mouse
Unigene: 201692 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.