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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
a) primary pulmonary hypertension
b) pulmonary venoocclusive diseas
General
bone morphogenetic protein receptor
glycoprotein
phosphoprotein
Properties
SIZE: entity length = 1038 aa
MW = 115 kD
COMPARTMENT: cellular membrane
STATE: active state
MOTIF: signal sequence {1-26}
cysteine residue {C34}
MODIFICATION: cysteine residue {C66}
N-glycosylation site {N55}
cysteine residue {C66}
MODIFICATION: cysteine residue {C34}
cysteine residue {C94}
MODIFICATION: cysteine residue {C117}
N-glycosylation site {N110}
cysteine residue {C117}
MODIFICATION: cysteine residue {C94}
N-glycosylation site {N126}
transmembrane domain {151-171}
kinase domain
SITE: 203-504
MOTIF: ATP-binding site
NAME: ATP-binding site
SITE: 209-217
ATP-binding site
NAME: ATP-binding site
SITE: 230-230
aspartate residue {D333}
serine-rich region {547-550}
MOTIF: serine residue (SEVERAL)
Ser phosphorylation site {S586}
threonine-rich region {610-618}
MOTIF: threonine residue (SEVERAL)
Ser phosphorylation site {S680}
Ser phosphorylation site {S681}
Ser phosphorylation site {S757}
Ser phosphorylation site {S863}
Database Correlations
OMIM correlations
UniProt Q13873
PFAM correlations
Entrez Gene 659
Kegg hsa:659
ENZYME 2.7.11.30
References
- UniProt :accession Q13873
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=BMPR2
Component-of
bone morphogenetic protein receptor complex