内皮素3抗体
规格：1mg/1ml
英文名： Endothelin 3
别名： ET 3; ET3; ET-3; PPET3; Preproendothelin 3; Preproendothelin3; Preproendothelin-3; Endothelin3; Endothelin-3; Endothelin 3; EDN3_HUMAN.
分子量： 9kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human ET-3
交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Rabbit,
细胞定位：分泌型蛋白
内皮素3抗体产品介绍：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 内皮素3抗体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 内皮素3抗体(Hirschsprung disease). 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 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
产内皮素3抗体品应用：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.
研究领域：肿瘤 **学 神经生物学 信号转导
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid