产品名称
ALK-1 Rabbit Polyclonal Antibody
别名
ACVRL1; ACVRLK1; ALK1; Serine/threonine-protein kinase receptor R3; SKR3; Activin receptor-like kinase 1; ALK-1; TGF-B superfamily receptor type I; TSR-I
蛋白名称
Serine/threonine-protein kinase receptor R3
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=94
Human Swissprot No.
P37023
Human Swissprot Link
http://www.uniprot.org/uniprotkb/P37023/entry
Mouse Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=11482
Mouse Swissprot No.
Q61288
Mouse Swissprot Link
http://www.uniprot.org/uniprot/Q61288
Rat Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=25237
Rat Swissprot Link
http://www.uniprot.org/uniprot/P80203
免疫原
The antiserum was produced against synthesized peptide derived from the N-terminal region of human ACVRL1. AA range:21-70
特异性
ALK-1 Polyclonal Antibody detects endogenous levels of ALK-1 protein.
稀释度
WB 1:500 - 1:2000. IHC-p: 1:100-300 ELISA: 1:20000.. IF 1:50-200
宿主
Polyclonal, Rabbit,IgG
背景介绍
This gene encodes a type I cell-surface receptor for the TGF-beta superfamily of ligands. It shares with other type I receptors a high degree of similarity in serine-threonine kinase subdomains, a glycine- and serine-rich region (called the GS domain) preceding the kinase domain, and a short C-terminal tail. The encoded protein, sometimes termed ALK1, shares similar domain structures with other closely related ALK or activin receptor-like kinase proteins that form a subfamily of receptor serine/threonine kinases. Mutations in this gene are associated with hemorrhagic telangiectasia type 2, also known as Rendu-Osler-Weber syndrome 2. [provided by RefSeq, Jul 2008],
细胞定位
Cell membrane ; Single-pass type I membrane protein .
信号通路
Cytokine-cytokine receptor interaction;TGF-beta;
功能
catalytic activity:ATP + [receptor-protein] = ADP + [receptor-protein] phosphate.,cofactor:Magnesium or manganese.,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.,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.,similarity:Belongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family. TGFB receptor subfamily.,similarity:Contains 1 GS domain.,similarity:Contains 1 protein kinase domain.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
