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.
Biological Context
Subcellular Location: Cell membrane (Single-pass type I membrane protein)
Disease Association: Telangiectasia, hereditary hemorrhagic, 2 (HHT2) : 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. [The disease is caused by variants affecting the gene represented in this entry]
Product Specifications
Recombinant Human Serine/threonine-protein kinase receptor R3 (ACVRL1), partial (Active) is a recombinant protein from Homo sapiens (Human), expressed in Baculovirus, covering amino acids 22-118aa, with C-terminal 6xHis-tagged tag, molecular weight 11.5KDa, purity Greater than 95% as determined by SDS-PAGE.. Suitable for ELISA and Western Blot applications. Explore more Kinase proteins →
Catalog number: BCACP-000250.
