Cdon and Boc: Two transmembrane proteins implicated in cell-cell communication.
Cdon and Boc, and their Drosophila homologues Ihog and Boi, are evolutionary conserved transmembrane glycoproteins belonging to a subgroup of the Immunoglobulin superfamily of cell adhesion molecules (CAMs). Initially isolated in vertebrates as CAMs that link cadherin function with MAPK signaling in myoblast differentiation, they have thereafter been shown to act as essential receptors for the Hedgehog (Hh) family of secreted proteins. They associate with both ligand and other Hh receptor components, including Ptch and Gas1, thus forming homo- and heteromeric complexes. In Drosophila, they are also involved in ligand processing and release from Hh producing cells. Cdon/Boc and Ihog/Boi can substitute one another and play redundant functions is some contexts. In addition, Boc, but not Cdon, mediates axon guidance information provided by Hh in specific neuronal populations, whereas mutations in the CDON cause holoprosencephaly, a human congenital anomaly defined by forebrain midline defects prominently associated with diminished Hh pathway activity.
Centro de Biología Molecular Severo Ochoa, CSIC-UAM and CIBER de Enfermedades Raras, c/ Nicolas Cabrera, 1, Madrid 28049, Spain.
Luisa
L
Sanchez-Arrones
Marcos
M
Cardozo
Francisco
F
Nieto-Lopez
Paola
P
Bovolenta
D000818:Animals; D002352:Carrier Proteins / Q000235:genetics / Q000378:metabolism; D015815:Cell Adhesion Molecules / Q000235:genetics / Q000378:metabolism*; D002450:Cell Communication; D029721:Drosophila Proteins / Q000235:genetics / Q000378:metabolism; D004331:Drosophila melanogaster; D018507:Gene Expression Regulation, Developmental; D053823:Hedgehog Proteins / Q000235:genetics / Q000378:metabolism; D016142:Holoprosencephaly / Q000235:genetics; D006801:Humans; D007074:Immunoglobulin G / Q000235:genetics / Q000378:metabolism*; D008562:Membrane Glycoproteins / Q000235:genetics / Q000378:metabolism; D009154:Mutation; D009474:Neurons / Q000166:cytology / Q000378:metabolism*; D011485:Protein Binding; D011956:Receptors, Cell Surface / Q000235:genetics / Q000378:metabolism*; D015398:Signal Transduction; D025521:Tumor Suppressor Proteins / Q000235:genetics / Q000378:metabolism*
C474334:BOC protein, human; C555140:Boi protein, Drosophila; C107286:CDON protein, human; D002352:Carrier Proteins; D015815:Cell Adhesion Molecules; D029721:Drosophila Proteins; D053823:Hedgehog Proteins; D007074:Immunoglobulin G; D008562:Membrane Glycoproteins; D011956:Receptors, Cell Surface; D025521:Tumor Suppressor Proteins; C510880:iHog protein, Drosophila