CDw293 BMPR1B (bone morphogenetic protein receptor type 1B),  ALK-6
Molecule TypeAntigen ExpressionMolecular Weight
Min / Max
Non-lineage Restricted Molecule
Type 1 glycoprotein
Bone Progenitor Cell
Mesenchymal Cell
Chondrocyte
Epithelial Cell
Heart
Kidney
50 / 50
58 / 58

Expression
CDw293 is expressed on epithelial, mesenchymal and bone progenitor cells, several embryonic tissues and chondrocytes.  Expression is in the heart and kidney.  

Structure
MOLECULAR FAMILY NAME: Belongs to the transmembrane serine/threonine kinase family.

CDw293 is a single-pass type-1 489 aa glycoprotein.  It contains a 13 aa signal sequence, an 113 extracellular domain which contains an activin receptor domain, a 22 aa transmembrane domain and an 354 aa intracellular cytoplasmic domain which contains a TGF β-like domain, a protein serine/threonine kinase domain and 2 ATP-binding sites.  It is a member of the bone morphogenetic protein receptor family of transmembrane serine/threonine kinases within TGFβ superfamily. CDw293 forms a heterodimer.  Magnesium or manganese is required as a cofactor for enzymatic activity.

MOLECULAR MASS

POST-TRANSCRIPTIONAL MODIFICATION: No information.

POST-TRANSLATIONAL MODIFICATION: No information.

Ligands
LIGANDS AND MOLECULE ASSOCIATED WITH CDw293

CDw293 binds bone morphogenetic protein (BMP)-2 and BMP-4.

Function
Proteins transduce their signals through the formation of teterameric complexes of 2 different types of serine (threonine) kinase receptors: CD292, type-1 receptors and CDw293, type II receptors.  Ligand binding activates SMAD transcriptional regulators.

BIOCHEMICAL ACTIVITY: No information.

DISEASE RELEVANCE AND FUNCTION OF CDw293 IN INTACT ANIMAL

CDw293 encodes a member of the bone morphogenetic protein receptor family of transmembrane serine/threonine kinases and is involved as an important regulator of endochondral bone formation and embryogenesis.  CDw293 plays a role in embryonic development inducing cell proliferation and differentiation in multiple embryonic tissues.  It is involved in kinase and regulates cartilage formation.  Bone development, chondrogenesis and retinal development are regulated by CD292 and CDw293 which appear to have overlapping functions.  Defects in CDw293 are the cause of osteochondrodysplasias (hereditary skeletal disorders) and brachydactyly type A2 (inherited malformations).  Mutations in this gene have been associated with pulmonary hypertension. 


Comments
MOLECULAR INTERACTIONS-
PROTEINS AND DNA ELEMENTS WHICH REGULATE TRANSCRIPTION OF CDw293: No information.

SUBSTRATES: No information.

ENZYMES WHICH MODIFY CDw293: No information.

ADDITIONAL INSIGHTS

For further information see ten Dijke, P. et al (1994) J. Biol. Chem. 269: 16985-16988.


Database accession numbers
AnimalPIRSWISSPROTEMGBL/GENBANK
 
HumanEntrezgene 658O00238
Antibodies

Revised June 25, 2008


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