Bone morphogenetic protein receptor, type 1

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Bone morphogenetic protein receptor, type IA

Ternary Complex Of Bmp-2 Bound To Bmpr-Ia-Ecd And Actrii-Ecd

Other names: Serine/threonine-protein kinase receptor R5, SKR5, Activin receptor-like kinase 3, ALK-3, CD292 antigen
Genetic data
Locus: Chr. 10 q22.3
Gene code: HUGO code:BMPR1A
Gene type: Protein coding
Protein Structure/Function
Protein length: 532 (Amino Acids)
Molecular Weight: 60198 (Da)
Structure: Complex Between Bmp-2 And Two Bmp Receptor Ia Ectodomains
Protein type: Receptor serine/threonine kinase
Functions: Receptor binding
Domains: TM domain S/T domain, GS domain
Motifs: SP motif
Other
Taxa expressing: Homo sapiens: homologs: many metazoan phyla
Cell types: many; prostate. cornea, brain
Subcellular localization: Plasma membrane
Pathway(s): TGF beta signaling pathway (KEGG) Cytokine-cytokine receptor interaction (KEGG)
Enzymatic Data
Catalytic activity: ATP + (receptor-protein) = ADP + receptor-protein phosphate
Cofactor(s): Magnesium or manganese
Receptor/Ligand data
Agonists: BMP2, BMP6, BMP7, GDF6
Antagonists: Noggin, Chordin
Medical/Biotechnological data
Diseases: Juvenile polyposis syndrome (JPS) Mendelian Inheritance in Man (OMIM) 174900; Cowden disease (CD) Mendelian Inheritance in Man (OMIM) 158350; Hereditary mixed polyposis syndrome 2 (HMPS2) Mendelian Inheritance in Man (OMIM) 610069

Bone morphogenetic protein receptor, type IB
Other names: CDw293 antigen
Genetic data
Gene code: HUGO code:BMPR1B
Protein Structure/Function
Protein type: Receptor serine/threonine kinase
Domains: TM domain S/T domain, GS domain
Other
Taxa expressing: Homo sapiens: homologs: many metazoan phyla
Cell types: many
Subcellular localization: Plasma membrane
Enzymatic Data
Catalytic activity: ATP + (receptor-protein) = ADP + receptor-protein phosphate
Cofactor(s): Magnesium or manganese
Medical/Biotechnological data
Diseases: Acromesomelic chondrodysplasia with genital anomalies Mendelian Inheritance in Man (OMIM) 609441; Brachydactyly type A2 (BDA2) Mendelian Inheritance in Man (OMIM) 112600


Bone morphogenetic protein type I receptors are single pass, type I transmembrane proteins. They belong to a class of receptor serine/threonine kinases that bind members of the TGF beta superfamily of ligands-the Bone morphogenetic proteins. There are two type I BMP receptors: BMPR1A and BPMR1B.

Contents

The BMPR1A receptor binds BMP2 and BMP4. BMP's repress WNT signaling to maintain stable stem cell populations. BMPR1A null mice died at embyonic day 8.0 without mesoderm specification, demonstrating its vital role in gastrulation[1]. It has been demonstrated in experiments using dominant negative BMPR1A chick embryos that BMPR1A plays a role in apoptosis and adipocyte development[1]. Using constitutively active forms of BMR1A it has been shown that it plays a role in cell differentiation[1]. Signals tranduced by the BMPR1A receptor are not essential for osteoblast formation or proliferation; however, BMPR1A is necessary for the extracellular matrix depostition by osteoblasts[1]. In the chick embryo, BMPR1A receptors are found in low levels in limb bud mesenchyme, a differing location to BMPR1B, supporting the differing roles they play in osteogenesis[2].

BMPR1A, SMAD4 and PTEN are responsible for Juvenile polyposis syndrome, juvenile intestinal polyposis and Cowdens' disease.

Identifiers
Symbol BMPR1A ACVRLK3
HUGO 1076
Entrez 657
OMIM 601299
RefSeq NM_004329
UniProt P36894
Other data

The BMPR1B receptor plays a role in the formation of middle and proximal phalanges [1]. In the chick embryo, it has been shown that BMPR1B is found in precartilaginous condensations[2]. BMPR1B is the major transducer of signlas in these condensations as demonstrated in experiments using constitutively active BMPR1B receptors[2]. BMPR1B is a more effective trasducer of GDF5 than BMPR1A[2]. Unlike BMPR1A null mice, which die at an early embryonic stage, BMPR1B null mice are viable[2].

Identifiers
Symbol BMPR1B
HUGO 1077
Entrez 658
OMIM 603248
RefSeq NM_001203
UniProt O00238
Other data

  1. ^ a b c d e Mishina, Y; Starbuck MW, Gentile MA, Fukuda T, Kasparcova V, Seedor JG, Hanks MC, Amling M, Pinero GJ, Harada S, Behringer RR (Jun 2004). "Bone morphogenetic protein type IA receptor signaling regulates postnatal osteoblast function and bone remodeling". J Biol Chem. 279 (26). Entrez PubMed 15090551. Retrieved on 2006-07-03. 
  2. ^ a b c d e Yoon, BS; Ovchinnikov DA, Yoshii I, Mishina Y, Behringer RR, Lyons KM. (Apr 2005). "Bmpr1a and Bmpr1b have overlapping functions and are essential for chondrogenesis in vivo". Proc Natl Acad Sci U S A. 102 (14). Entrez PubMed 15781876. Retrieved on 2006-07-03. 
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