Anti-ALK1

ALK-1 or Activin receptor-like kinase-1 is a type I cell surface receptor for the TGF-beta (transforming growth factor-beta) super family of proteins. It is expressed predominantly in endothelial cells with moderate expression in smooth muscle cells, myofibroblast, hepatic stellate cells, chondrocytes, monocytes, myoblasts, macrophages and fibroblasts. ALK-1 plays a pivotal role in vascular remodelling and angiogenesis. More specifically its key function is in arterial/venous differentiation during embryonic vascular development. Mutations in ALK-1 gene (12q11-14) causes HHT2 (Hereditary Haemorrhagic Telangiectasia type 2), an autosomal dominant multisystem vascular dysplasia caused by a haploinsufficiency mechanism. Protein 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. [The UniProt Consortium]