serine/threonine protein kinase receptor R3; SKR3; activin receptor-like kinase 1; ALK-1; TGF-B superfamily receptor type I; TSR-I (ACVRL1 ACVRLK1 ALK1)
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Function
- receptor for TGF-beta
- may bind activin as well
- on ligand binding, forms a receptor complex consisting of 2 type II & 2 type I transmembrane serine/threonine kinases
- type II receptors phosphorylate & activate type I receptors which autophosphorylate, then bind & activate SMAD transcriptional regulators
Cofactor: Mg+2 or Mn+2 (putative)
Structure
- belongs to the protein kinase superfamily, TKL Ser/Thr protein kinase family, TGFB receptor subfamily
- contains 1 GS domain
- contains 1 protein kinase domain
Compartment
membrane
Pathology
- defects in ACVRL1 are the cause of Osler-Weber-Rendu syndrome 2