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Anti-ACVL1 Antibody

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产品名称: Anti-ACVL1 Antibody
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Anti-ACVL1 Antibody


Anti-ACVL1 Antibody  的详细介绍
Name: Anti-ACVL1 Antibody
See all ACVL1 primary antibodies
Description: Rabbit polyclonal antibody to ACVL1
Specificity: The antibody detects endogenous levels of total ACVL1 protein.
Applications: WB, IHC, IF
Reactivity: Human, Mouse, Rat
Immunogen: Synthesized peptide derived from internal of human ACVL1.
Host: Rabbit
Clonality: Polyclonal
Conjugate: Unconjugated
Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Concentration: 1.0mg / ml
Formulation: Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
Storage: Store at -20?C
Function: Type I receptor for TGF-beta family ligands BMP9/GDF2 and BMP10 and important regulator of normal blood vessel development. 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. May bind activin as well.
Involvement in Disease: Telangiectasia, hereditary hemorrhagic, 2: A multisystemic vascular dysplasia leading to dilation of permanent blood vessels and arteriovenous malformations of skin, mucosa, and viscera. The disease is characterized by recurrent epistaxis and gastro-intestinal hemorrhage. Visceral involvement includes arteriovenous malformations of the lung, liver, and brain.
Sequence Similarities: Belongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family. TGFB receptor subfamily.
Cellular Location: Cell membrane.
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