Human ALK-1 / ACVRL1 Protein (Fc Tag)

ACVRLK1,ALK-1,ALK1,HHT,HHT2,ORW2,SKR3,TSR-I

100ug (NPP1888) Please inquiry

Catalog Number	P10066-H02H
Organism Species	Human
Host	Human Cells
Synonyms	ACVRLK1,ALK-1,ALK1,HHT,HHT2,ORW2,SKR3,TSR-I
Molecular Weight	The mature recombinant human ALK1/Fc chimera is a disulfide-linked homodimeric protein. The reduced monomer comprises 335 amino acids and has a predicted molecular mass of 37.4 kDa. As a result of glycosylation, the recombinant monomer migrates as an approximately 45-50 kDa protein in SDS-PAGE under reducing conditions.
predicted N	Asp 22
SDS-PAGE
Purity	> 97 % as determined by SDS-PAGE
Protein Construction	A DNA sequence encoding the N-terminal segment (Met 1-Gln 118) of the extracellular domain of human ALK1 (NP_000011.2) pro-protein was fused with the Fc region of human IgG1 at the C-terminus.
Bio-activity	1. Measured by its ability to bind Human ENG-Fc (P10149-H02H) in functional Elisa. 2. Measured by its ability to latent TGFB1-His (P10804-H08H) in functional Elisa. 3. Measured by its ability to mouse ENG-His (P50407-M08H) in functional Elisa. 4. Measured by its ability to inhibit BMP9 induced alkaline phosphatase production by MC3T3E1 mouse chondrogenic cells. David, L. et al. (2007) Blood 109:1953. The ED₅₀ for this effect is typically 5-15 ng/mL in the presence of 2 ng/mL of recombiant human BMP9.
Research Area	Cancer \|Invasion microenvironment \|Angiogenesis \|Adhesion Molecules in Angiogenesis \|Extracellular Matrix \|Structures \|
Formulation	Lyophilized from sterile PBS, pH 7.4 1. Normally 5 % - 8 % trehalose and mannitol are added as protectants before lyophilization. Specific concentrations are included in the hardcopy of COA.
Background	Activin A receptor, type II-like 1 (ACVRL1), also known as ALK-1 (activin receptor-like kinase 1), is an endothelial-specific type I receptor of the TGF-beta (transforming growth factor beta) receptor family of ligands. On ligand binding, a heteromeric receptor complex forms consisting of two type II and two type I transmembrane serine/threonine kinases. ACVRL1 protein is expressed in certain blood vessels of kidney, spleen, heart and intestine, serving as an important role during vascular development. Mutations in ACVRL1 gene are associated with hemorrhagic telangiectasia type 2, also known as Rendu-Osler-Weber syndrome 2 and vascular disease.
Reference	French Rendu-Osler network,et al. (2004) Molecular screening of ALK1/ACVRL1 and ENG genes in hereditary hemorrhagic telangiectasia in France. Hum Mutat. 23(4): 289-299. Simon M, et al. (2006) Association of a polymorphism of the ACVRL1 gene with sporadic arteriovenous malformations of the central nervous system. J Neurosurg. 104(6): 945-9. Argyriou L, et al. (2006) Novel mutations in the ENG and ACVRL1 genes causing hereditary hemorrhagic teleangiectasia. Int J Mol Med. 17(4):655-9.