Molecular basis of ALK1-mediated signalling by BMP9/BMP10 and their prodomain-bound forms

Nat Commun. 2020 Apr 1;11(1):1621. doi: 10.1038/s41467-020-15425-3.

Abstract

Activin receptor-like kinase 1 (ALK1)-mediated endothelial cell signalling in response to bone morphogenetic protein 9 (BMP9) and BMP10 is of significant importance in cardiovascular disease and cancer. However, detailed molecular mechanisms of ALK1-mediated signalling remain unclear. Here, we report crystal structures of the BMP10:ALK1 complex at 2.3 Å and the prodomain-bound BMP9:ALK1 complex at 3.3 Å. Structural analyses reveal a tripartite recognition mechanism that defines BMP9 and BMP10 specificity for ALK1, and predict that crossveinless 2 is not an inhibitor of BMP9, which is confirmed by experimental evidence. Introduction of BMP10-specific residues into BMP9 yields BMP10-like ligands with diminished signalling activity in C2C12 cells, validating the tripartite mechanism. The loss of osteogenic signalling in C2C12 does not translate into non-osteogenic activity in vivo and BMP10 also induces bone-formation. Collectively, these data provide insight into ALK1-mediated BMP9 and BMP10 signalling, facilitating therapeutic targeting of this important pathway.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Activin Receptors, Type II / chemistry
  • Activin Receptors, Type II / metabolism*
  • Animals
  • Binding Sites
  • Bone Morphogenetic Proteins / chemistry
  • Bone Morphogenetic Proteins / metabolism*
  • Bone and Bones / chemistry
  • Bone and Bones / metabolism
  • Cell Line
  • Crystallography, X-Ray
  • Endothelial Cells / metabolism
  • Growth Differentiation Factor 2 / chemistry
  • Growth Differentiation Factor 2 / metabolism*
  • Humans
  • Ligands
  • Male
  • Mice
  • Mice, Inbred C57BL
  • Models, Molecular
  • Protein Conformation
  • Protein Domains
  • Signal Transduction / physiology*
  • Transforming Growth Factor beta / metabolism

Substances

  • BMP10 protein, human
  • Bone Morphogenetic Proteins
  • GDF2 protein, human
  • Growth Differentiation Factor 2
  • Ligands
  • Transforming Growth Factor beta
  • ACVRL1 protein, human
  • Activin Receptors, Type II