Endothelin 3,内皮素3抗体

产品编号HZ-5897R
英文名称Endothelin 3
中文名称内皮素3抗体
别 名ET 3; ET3; ET-3; PPET3; Preproendothelin 3; Preproendothelin3; Preproendothelin-3; Endothelin3; Endothelin-3; Endothelin 3; EDN3_HUMAN.
说 明 书0.1ml 0.2ml
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,
Endothelin 3,内皮素3抗体产品应用ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量9kDa
细胞定位分泌型蛋白
性 状Lyophilized or Liquid
浓 度1mg/1ml
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.
Endothelin 3,内皮素3抗体PubMedPubMed
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).

Endothelin 3,内皮素3抗体Similarity:
Belongs to the endothelin/sarafotoxin family.

Gene ID:
1908

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

Endothelin 3,内皮素3抗体Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.