GPC3

Cell surface heparan sulfate proteoglycans are composed of a membrane-associated protein core substituted with a variable number of heparan sulfate chains. Members of the glypican-related integral membrane proteoglycan family (GRIPS) contain a core protein anchored to the cytoplasmic membrane via a glycosyl phosphatidylinositol linkage. These proteins may play a role in the control of cell division and growth regulation. The protein encoded by this gene can bind to and inhibit the dipeptidyl peptidase activity of CD26, and it can induce apoptosis in certain cell types. Deletion mutations in this gene are associated with Simpson-Golabi-Behmel syndrome, also known as Simpson dysmorphia syndrome. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Sep 2009]

Glypican 3

Cell surface proteoglycan that bears heparan sulfate (PubMed:14610063).

Negatively regulates the hedgehog signaling pathway when attached via the GPI-anchor to the cell surface by competing with the hedgehog receptor PTC1 for binding to hedgehog proteins (By similarity).

Binding to the hedgehog protein SHH triggers internalization of the complex by endocytosis and its subsequent lysosomal degradation (By similarity).

Positively regulates the canonical Wnt signaling pathway by binding to the Wnt receptor Frizzled and stimulating the binding of the Frizzled receptor to Wnt ligands (PubMed:16227623, PubMed:24496449).

Positively regulates the non-canonical Wnt signaling pathway (By similarity).

Binds to CD81 which decreases the availability of free CD81 for binding to the transcriptional repressor HHEX, resulting in nuclear translocation of HHEX and transcriptional repression (By similarity).

Inhibits the dipeptidyl peptidase activity of DPP4 (PubMed:17549790).

Plays a role in limb patterning and skeletal development by controlling the cellular response to BMP4 (By similarity).

Modulates the effects of growth factors BMP2, BMP7 and FGF7 on renal branching morphogenesis (By similarity).

Required for coronary vascular development (By similarity).

Plays a role in regulating cell movements during gastrulation (By similarity).

Anatomical structure morphogenesis Source: ProtInc
Anterior/posterior axis specification Source: Ensembl
Body morphogenesis Source: Ensembl
Bone mineralization Source: Ensembl
Branching involved in ureteric bud morphogenesis Source: Ensembl
Cell migration Source: GO_Central
Cell migration involved in gastrulation Source: UniProtKB
Cell proliferation involved in kidney development Source: UniProtKB
Cell proliferation involved in metanephros development Source: Ensembl
Coronary vasculature development Source: UniProtKB
Embryonic hindlimb morphogenesis Source: Ensembl
Lung development Source: Ensembl
Mesenchymal cell proliferation involved in ureteric bud development Source: UniProtKB
Mesonephric duct morphogenesis Source: UniProtKB
Negative regulation of canonical Wnt signaling pathway Source: Ensembl
Negative regulation of epithelial cell proliferation Source: Ensembl
Negative regulation of growth Source: Ensembl
Negative regulation of peptidase activity Source: UniProtKB-KW
Negative regulation of smoothened signaling pathway Source: UniProtKB
Osteoclast differentiation Source: Ensembl
Positive regulation of BMP signaling pathway Source: Ensembl
Positive regulation of canonical Wnt signaling pathway Source: UniProtKB
Positive regulation of endocytosis Source: UniProtKB
Positive regulation of glucose import Source: Ensembl
Positive regulation of protein catabolic process Source: UniProtKB
Positive regulation of smoothened signaling pathway Source: Ensembl
Positive regulation of Wnt signaling pathway, planar cell polarity pathway Source: Ensembl
Regulation of canonical Wnt signaling pathway Source: UniProtKB
Regulation of non-canonical Wnt signaling pathway Source: UniProtKB
Regulation of protein localization to membrane Source: GO_Central
Response to bacterium Source: Ensembl

Cell membrane

Simpson-Golabi-Behmel syndrome 1 (SGBS1):
A condition characterized by pre- and postnatal overgrowth (gigantism), facial dysmorphism and a variety of inconstant visceral and skeletal malformations. Characteristic dysmorphic features include macrocephaly with coarse, distinctive facies with a large protruding jaw, broad nasal bridge and cleft palate. Cardiac defects are frequent.

O-glycosylated; contains heparan sulfate.
Cleaved intracellularly by a furin-like convertase to generate 2 subunits, alpha and beta, which remain associated through disulfide bonds and are associated with the cell surface via the GPI-anchor (PubMed:14610063). This processing is essential for its role in inhibition of hedgehog signaling (PubMed:25653284). A second proteolytic event may result in cleavage of the protein on the cell surface, separating it from the GPI-anchor and leading to its shedding from the cell surface (PubMed:14610063).