|CDw293||BMPR1B (bone morphogenetic protein receptor type 1B), ALK-6|
|Molecule Type||Antigen Expression||Molecular Weight|
Min / Max
|Non-lineage Restricted Molecule|
Type 1 glycoprotein
|Bone Progenitor Cell|
|50 / 50|
58 / 58
|CDw293 is expressed on epithelial, mesenchymal and bone progenitor cells, several embryonic tissues and chondrocytes. Expression is in the heart and kidney. |
|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.
POST-TRANSCRIPTIONAL MODIFICATION: No information.
POST-TRANSLATIONAL MODIFICATION: No information.
|LIGANDS AND MOLECULE ASSOCIATED WITH CDw293|
CDw293 binds bone morphogenetic protein (BMP)-2 and BMP-4.
|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.
PROTEINS AND DNA ELEMENTS WHICH REGULATE TRANSCRIPTION OF CDw293: No information.
SUBSTRATES: No information.
ENZYMES WHICH MODIFY CDw293: No information.
For further information see ten Dijke, P. et al (1994) J. Biol. Chem. 269: 16985-16988.
Database accession numbers
Revised June 25, 2008