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Rabbit Anti-ACVRL1/Gold Conjugated antibody (bs-6020R-Gold)
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说 明 书: 100ul(10nm  15nm  35nm
100ul/2980.00元
大包装/询价
产品编号 bs-6020R-Gold
英文名称 Anti-ACVRL1/Gold
中文名称 胶体金标记的激活素受体样激酶1抗体
别    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.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul(10nm  15nm  35nm
研究领域 心血管  细胞生物  免疫学  信号转导  转录调节因子  激酶和磷酸酶  细胞表面分子  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human, Mouse, Rat, Dog, Pig, Horse, )
产品应用 IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 53kDa
性    状 Lyophilized or Liquid
浓    度 0.4mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ACVRL1/ALK1
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300.
保存条件 Store at 2-8 ºC for 3-6 months. Avoid repeated freeze/thaw cycles.
产品介绍 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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