ETBR,内皮素B受体抗体

产品编号HZ-2363R
英文名称ETBR
中文名称内皮素B受体抗体
别 名ABCDS; Ednra; Ednrb; EDNRB_HUMAN; Endothelin B receptor; Endothelin B receptor precursor; Endothelin receptor Non selective type; Endothelin receptor non-selective type; Endothelin receptor type B; ET B; ET-B; ET-BR; ETB; ETRB; Hirschsprung disease 2; HSCR; HSCR2; EDNRB; HSCR; WS4A.
说 明 书0.1ml 0.2ml
研究领域心血管 免疫学 神经生物学 生长因子和激素
抗体来源Rabbit
克隆类型Polyclonal
交叉反应 Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit,
ETBR,内皮素B受体抗体产品应用WB=1:100-500 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50kDa
细胞定位细胞膜
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human EDNRB
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.
ETBR,内皮素B受体抗体PubMedPubMed
产品介绍background:
Endothelin receptor type B (ETRB) is a G protein-coupled receptor that activates a phosphatidylinoitol-calcium second messenger system. Its ligand, endothelin, consists of a family of three potent vasoactive peptides: ET1, ET2, and ET3. A splice variant (SVR), differing in the intracellular C terminal domain, has been described for this receptor. Both WT and the variant bind ET1. However, they exhibit different responses upon binding - suggesting that these two variants are functionally distinct.

Function:
Non-specific receptor for endothelin 1, 2, and 3. Mediates its action by association with G proteins that activate a phosphatidylinositol-calcium second messenger system.

Subcellular Location:
Cell membrane; Multi-pass membrane protein.

Tissue Specificity:
Expressed in placental stem villi vessels, but not in cultured placental villi smooth muscle cells.

Post-translational modifications:
Palmitoylation of Cys-402 was confirmed by the palmitoylation of Cys-402 in a deletion mutant lacking both Cys-403 and Cys-405.

ETBR,内皮素B受体抗体DISEASE:
Defects in EDNRB are a cause of Waardenburg syndrome type 4A (WS4A) [MIM:277580]; also known as Waardenburg-Shah syndrome. WS4A 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).
Defects in EDNRB are the cause of Hirschsprung disease type 2 (HSCR2) [MIM:600155]; also known as aganglionic megacolon (MGC). HSCR2 is a congenital disorder characterized by absence of enteric ganglia along a variable length of the intestine. It is the most common cause of congenital intestinal obstruction. Early symptoms range from complete acute neonatal obstruction, characterized by vomiting, abdominal distention and failure to pass stool, to chronic constipation in the older child.
Defects in EDNRB are the cause of ABCD syndrome (ABCDS) [MIM:600501]. ABCD syndrome is an autosomal recessive syndrome characterized by albinism, black lock at temporal occipital region, bilateral deafness, aganglionosis of the large intestine and total absence of neurocytes and nerve fibers in the small intestine.

Similarity:
Belongs to the G-protein coupled receptor 1 family. Endothelin receptor subfamily. EDNRB sub-subfamily.

Gene ID:
1910

Database links:
Entrez Gene: 408082 Chicken
Entrez Gene: 281750 Cow
Entrez Gene: 1910 Human
Entrez Gene: 13618 Mouse
Entrez Gene: 100009477 Rabbit
Entrez Gene: 50672 Rat
Omim: 131244 Human
SwissProt: P28088 Cow
SwissProt: P24530 Human
SwissProt: P48302 Mouse
SwissProt: P35463 Pig
SwissProt: Q9N0W7 Rabbit
SwissProt: P21451 Rat
Unigene: 487 Cow
Unigene: 82002 Human
Unigene: 229532 Mouse
Unigene: 27603 Pig
Unigene: 6857 Rabbit
Unigene: 11412 Rat

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