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FLASH GENE
Symbol GPC3 contributors: mct - updated : 25-10-2013
HGNC name glypican 3
HGNC id 4451
Corresponding disease
SGBS Simpson-Golabi-Behmel syndrome
Location Xq26.2      Physical location : 132.669.775 - 133.119.673
Synonym name
  • glypican proteoglycan 3
  • intestinal protein OCI-5
  • heparan sulphate proteoglycan
  • secreted glypican-3
  • Synonym symbol(s) DGSX, OCI-5, GTR2-2, SDYS, SGBS1, SGB, MXR7, SGBS
    DNA
    TYPE functioning gene
    SPECIAL FEATURE
    text adjacent to GPC4, resulting of gene duplication
    STRUCTURE 449.90 kb     9 Exon(s)
    10 Kb 5' upstream gene genomic sequence study
    regulatory sequence Binding site   transcription factor
    text structure NFY and Sp1 binding sites
    MAPPING cloned Y linked N status confirmed
    Physical map
    OR7L1P Xq26.2 olfactory receptor, family 7, subfamily L, member 1 pseudogene OR2AF2P Xq26.2 olfactory receptor, family 2, subfamily AF, member 2 pseudogene LOC389886 X similar to hypothetical protein MGC15416 MST4 Xq26.1 similar to hypothetical protein MGC15416 LOC90167 Xq26.2 hypothetical protein LOC90167 LOC392543 X similar to hypothetical protein FLJ10842 RAP2C Xq25 RAP2C, member of RAS oncogene family MBNL3 Xq26.2 muscleblind-like 3 (Drosophila) HS6ST2 Xq26.1-q26.2 heparan sulfate 6-O-sulfotransferase 2 USP26 Xq26.2 ubiquitin specific protease 26 LOC51270 Xq26.2 E2F-like protein GPC4 Xq26.1 glypican 4 GPC3 Xq26.1 glypican 3 LOC347475 Xq26.3 hypothetical gene supported by BC017958 LOC392544 X hypothetical gene supported by NM_152729 PHF6 Xq26 PHD finger protein 6 HPRT1 Xq26.1 hypoxanthine phosphoribosyltransferase 1 (Lesch-Nyhan syndrome) MGC16121 Xq26.3 hypothetical protein MGC16121 PLAC1 Xq26 placenta-specific 1 LOC159090 Xq26.3 similar to hypothetical protein MGC17347 LOC159091 Xq26.3 hypothetical protein BC017868 DJ473B4 Xq26.3 hypothetical protein dJ473B4 LOC340581 Xq26.3 hypothetical protein LOC340581 LOC392545 X similar to high mobility group protein homolog HMG4 LOC389887 X similar to RIKEN cDNA 1110012O05 CXX1 Xq26 CAAX box 1 DKFZP564B147 Xq26.3 DKFZP564B147 protein LOC159049 Xq26.3 hypothetical LOC159049 FLJ20527 Xq26.3 hypothetical protein FLJ20527 LOC392546 X similar to hypothetical protein FLJ20527 LOC389888 X similar to hypothetical protein FLJ20527 LOC389889 X hypothetical gene supported by BC061642 ZNF75 Xq26 zinc finger protein 75 (D8C6) FLJ23614 Xq26.3 hypothetical protein FLJ23614
    regionally located juxtaposed to GPC4
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    8 - 2329 65.4 580 - 2008 18785116
    7 - 2167 - 526 - 2008 18785116
    8 - 2281 - 564 - 2008 18785116
    9 - 2398 - 603 - 2008 18785116
    EXPRESSION
    Type ubiquitous
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    blood / hematopoieticspleen    
    Cardiovascularheart   highly
    Digestiveliver   highly
     mouthtongue  highly
    Endocrinepancreas    
    Lymphoid/Immunethymus    
    Reproductivefemale systemovary   
     female systemplacenta  highly
    Respiratorylung    
    Urinarykidney    
    tissue
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    Epithelialsecretoryglandularendocrine 
    Lymphoid    
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    physiological period fetal
    Text spleen, placenta, peak expression during development and down-regulated in all tissues after birth (Boily 2007)
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • a glycosyl phosphatidylinositol anchor, with 13 cysteines at conserved position
  • HOMOLOGY
    interspecies homolog to murine Gpc3
    Homologene
    FAMILY
  • glypican family
  • CATEGORY tumor suppressor
    SUBCELLULAR LOCALIZATION extracellular
        plasma membrane
        intracellular
    intracellular,cytoplasm,organelle,lumen
    intracellular,cytoplasm,organelle,Golgi
    intracellular,cytoplasm,organelle,lysosome
    text
  • attached to the cell surface by a glycosylphosphatidylinositol anchor
  • basic FUNCTION
  • heparan sulfate proteoglycans that are anchored to the cell membrane by a glycosyl-phosphatidylinositol protein
  • involved in growth control in embryonic mesodermal tissues
  • putative modulator of IGF2, juxtaposed to GPC4, with tumor suppressor activity in malignant mesothelioma or other tumors
  • plays a negative role in cell proliferation and an apoptosis-inducing role in specific tissues
  • may play roles in glucose transport through SLC2A4
  • may play a role in insulin stimulated glucose uptake
  • cell surface proteoglycans, with expression highly tissue- and stage-specific during human development
  • confers oncogenecity through the interaction between IGF2 and its receptor, and the subsequent activation of the IGF-signaling pathway
  • plays a critical role in promoting hepatocellular carcinoma cell growth and differentiation
  • heparan sulfate proteoglycan that has an important role in cell growth and differentiation, and its function in tumorigenesis is tissue-dependent
  • down-regulates cell proliferation by binding to HH and down-regulating the HH signaling pathway and binding with CD81, thus making it unavailable to bind to HHEX and causing its nuclear translocation
  • CELLULAR PROCESS cell life, cell death/apoptosis
    cell life, antiapoptosis
    PHYSIOLOGICAL PROCESS development
    PATHWAY
    metabolism
    signaling
    a component
  • proteoglycan putative constituent of extracellular matrix complexing with IGF2
  • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • SLC2A4-binding protein (GPC3 and SLC2A4 co-localizes at the plasma membrane in response to insulin)
  • binds to and inhibits the dipeptidyl peptidase activity of DPP4
  • plays a role in glucose transport by interacting with SLC2A1
  • is a transcriptional target of MYC and the expression of MYC is also regulated by GPC3, thus forming a positive feedback signaling loop
  • CD81 is a binding partner for GPC3, and its expression and co-localization with GPC3 increased at the end of hepatocyte proliferation
  • cell & other
    REGULATION
    Other regulated by NFYA, NFYB, NFYC and others cell type-specific factors (may play an important role in the regulation of GPC3)
    ASSOCIATED DISORDERS
    corresponding disease(s) SGBS
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    tumoral     --low  
    in mesothelioma, breast and ovarian cancer
    tumoral     --over  
    in hepatocellular carcinoma (HCC), neuroblastoma and Wilm's tumor cells
    tumoral     --low  
    in intrahepatic chanlangiocarcinoma
    Susceptibility
    Variant & Polymorphism
    Candidate gene
  • specific immunomarker for HCC that can be used to distinguish HCC from benign hepatocellular mass lesions, particularly hepatocellular adenoma , but combination of AFP and GPC3 can elevate the sensitivity of diagnosis
  • may have future application as a biomarker for rhabdomyosarcomas
  • Marker
    Therapy target
    SystemTypeDisorderPubmed
    cancerdigestiveliver
    anti-glypican 3 antibody against the C-terminal 30-kDa fragment such as GC33 are useful in therapy targeting hepatocellular carcinoma
    cancermuscle 
    may be a therapeutic target in rhabdomyosarcomas (RMS), the most common paediatric soft tissue sarcomas
    ANIMAL & CELL MODELS
  • Gpc3-deficient mice display a high incidence of congenital cardiac malformations like ventricular septal defects, common atrioventricular canal and double outlet right ventricle