bone morphogenetic protein receptor type-2; bone morphogenetic protein receptor type II; BMP type II receptor; BMPR-II (BMPR2, PPH1)

Function

• 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
• binds to BMP-7, BMP-2 &, less efficiently, BMP-4
• binding is weak but enhanced by the presence of type I receptors for BMPs

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 protein kinase domain

Compartment

membrane

Expression

highly expressed in heart & liver

Pathology

• defects in BMPR2 are the cause of
  • primary pulmonary hypertension
  • pulmonary venoocclusive diseas

More general terms

• bone morphogenetic protein receptor
• phosphoprotein
• glycoprotein

Database

• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:659
• OMIM: https://mirror.omim.org/entry/178600
• OMIM: https://mirror.omim.org/entry/265450
• OMIM: https://mirror.omim.org/entry/600799
• UniProt: http://www.uniprot.org/uniprot/Q13873.html
• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=659