Bone Morphogenetic Protein Receptor 2 (BMPR2)

BMPRII; BMPR-II; BRK3; T-ALK; BMPR3; PPH1; Primary Pulmonary Hypertension 1; BMP type II receptor

SPN	W690
HGNC	1078
MGI	1095407
PubMed	16429403
RGD	71082
UniProt	Q13873
Wiki	BMPR2

Bone morphogenetic protein receptor type II or BMPR2 is a serine/threonine receptor kinase. It binds Bone morphogenetic proteins, members of the TGF beta superfamily of ligands. BMPs are involved in host of cellular functions including osteogenesis, cell growth and cell differentiation. Signaling in the BMP pathway begins with the binding of a BMP to the type II receptor. This causes the recruitment of a BMP type I receptor, which it phosphorylates. The Type I receptor phosphorylates an R-SMAD a transcriptional regulator.Unlike the TGFβ type II receptor, which has a high affinity for TGF-β1, BMPR2 does not have a high affinity for BMP-2, BMP-7 and BMP-4, unless it is co-expressed with a type I BMP receptor. In TGF beta signaling all of the receptors exist in homodimers before ligand binding.

Reference

"Human type II receptor for bone morphogenic proteins (BMPs): extension of the two-kinase receptor model to the BMPs."
Mol. Cell. Biol. 15:3479-3486(1995) [PubMed] [Europe PMC] [Abstract]

"Cloning and characterization of a human type II receptor for bone morphogenetic proteins."
Proc. Natl. Acad. Sci. U.S.A. 92:7632-7636(1995) [PubMed] [Europe PMC] [Abstract]

"Identification of a human type II receptor for bone morphogenetic protein-4 that forms differential heteromeric complexes with bone morphogenetic protein type I receptors."
J. Biol. Chem. 270:22522-22526(1995) [PubMed] [Europe PMC] [Abstract]

"Cloning of a novel type II serine/threonine kinase receptor through interaction with the type I transforming growth factor-beta receptor."
J. Biol. Chem. 270:5625-5630(1995) [PubMed] [Europe PMC] [Abstract]

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Nature 434:724-731(2005) [PubMed] [Europe PMC] [Abstract]

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Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]

"Kinase-selective enrichment enables quantitative phosphoproteomics of the kinome across the cell cycle."
Mol. Cell 31:438-448(2008) [PubMed] [Europe PMC] [Abstract]

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Mol. Cell. Proteomics 8:1751-1764(2009) [PubMed] [Europe PMC] [Abstract]

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"An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."
J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]

"Crystal structure of the BMPR2 kinase domain."
Submitted (FEB-2009) to the PDB data bank

"Familial primary pulmonary hypertension (gene PPH1) is caused by mutations in the bone morphogenetic protein receptor-II gene."
Am. J. Hum. Genet. 67:737-744(2000) [PubMed] [Europe PMC] [Abstract]

"Sporadic primary pulmonary hypertension is associated with germline mutations of the gene encoding BMPR-II, a receptor member of the TGF-beta family."
J. Med. Genet. 37:741-745(2000) [PubMed] [Europe PMC] [Abstract]

"Heterozygous germline mutations in BMPR2, encoding a TGF-beta receptor, cause familial primary pulmonary hypertension."
Nat. Genet. 26:81-84(2000) [PubMed] [Europe PMC] [Abstract]

"BMPR2 haploinsufficiency as the inherited molecular mechanism for primary pulmonary hypertension."
Am. J. Hum. Genet. 68:92-102(2001) [PubMed] [Europe PMC] [Abstract]

"BMPR2 germline mutations in pulmonary hypertension associated with fenfluramine derivatives."
Eur. Respir. J. 20:518-523(2002) [PubMed] [Europe PMC] [Abstract]

"Pulmonary veno-occlusive disease caused by an inherited mutation in bone morphogenetic protein receptor II."
Am. J. Respir. Crit. Care Med. 167:889-894(2003) [PubMed] [Europe PMC] [Abstract]

"BMPR2 mutations have short lifetime expectancy in primary pulmonary hypertension."
Hum. Mutat. 26:119-124(2005) [PubMed] [Europe PMC] [Abstract]

"Mutations of the TGF-beta type II receptor BMPR2 in pulmonary arterial hypertension."
Hum. Mutat. 27:121-132(2006) [PubMed] [Europe PMC] [Abstract]

"Patterns of somatic mutation in human cancer genomes."
Nature 446:153-158(2007) [PubMed] [Europe PMC] [Abstract]

"Functional changes in pulmonary arterial endothelial cells associated with BMPR2 mutations."
PLoS ONE 9:E106703-E106703(2014) [PubMed] [Europe PMC] [Abstract]