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Rabbit Anti-ACVRL1/AP Conjugated antibody (bs-6020R-AP)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-6020R-AP
英文名稱 Rabbit Anti-ACVRL1/AP Conjugated antibody
中文名稱 堿性磷酸酶(AP)標記的激活素受體樣激酶1抗體
別    名 Activin A receptor; Activin A receptor type II like 1; Activin receptor like kinase 1; Activin receptor-like kinase 1; ACVL1_HUMAN; ACVRL1; ACVRLK1; ALK-1; ALK1; HHT; HHT2; ORW2; Osler Rendu Weber syndrome 2; Serine/threonine protein kinase receptor R3; Serine/threonine-protein kinase receptor R3; SKR3; TGF B superfamily receptor type I; TGF-B superfamily receptor type I; TSR-I; TSR1.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 心血管  細胞生物  免疫學  信號轉導  轉錄調節因子  激酶和磷酸酶  細胞表面分子  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Dog, Pig, Horse, )
產品應用 WB=1:50-200 IHC-P=1:50-200 IHC-F=1:50-200 ICC=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 53kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ACVRL1/ALK1
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產品介紹 background:
On ligand binding, forms a receptor complex consisting of two type II and two type I transmembrane serine/threonine kinases. Type II receptors phosphorylate and activate type I receptors which autophosphorylate, then bind and activate SMAD transcriptional regulators. Receptor for TGF-beta. May bind activin as well.

Function:
On ligand binding, forms a receptor complex consisting of two type II and two type I transmembrane serine/threonine kinases. Type II receptors phosphorylate and activate type I receptors which autophosphorylate, then bind and activate SMAD transcriptional regulators. Receptor for TGF-beta. May bind activin as well.

Subcellular Location:
Membrane.

DISEASE:
Defects in ACVRL1 are the cause of hereditary hemorrhagic telangiectasia type 2 (HHT2) [MIM:600376]; also known as Osler-Rendu-Weber syndrome 2 (ORW2). HHT2 is an autosomal dominant multisystemic vascular dysplasia, characterized by recurrent epistaxis, muco-cutaneous telangiectases, gastro-intestinal hemorrhage, and pulmonary, cerebral and hepatic arteriovenous malformations; all secondary manifestations of the underlying vascular dysplasia.

Similarity:
Belongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family. TGFB receptor subfamily.
Contains 1 GS domain.
Contains 1 protein kinase domain.

Database links:
 

 

Entrez Gene: 94 Human

Omim: 601284 Human

SwissProt: P37023 Human

Unigene: 591026 Human



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