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Protein

Glypican-3

Gene

GPC3

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Cell surface proteoglycan that bears heparan sulfate. Inhibits the dipeptidyl peptidase activity of DPP4. May be involved in the suppression/modulation of growth in the predominantly mesodermal tissues and organs. May play a role in the modulation of IGF2 interactions with its receptor and thereby modulate its function. May regulate growth and tumor predisposition.1 Publication

GO - Molecular functioni

  • peptidyl-dipeptidase inhibitor activity Source: UniProtKB

GO - Biological processi

Keywordsi

Molecular functionProtease inhibitor

Enzyme and pathway databases

ReactomeiR-HSA-1971475 A tetrasaccharide linker sequence is required for GAG synthesis
R-HSA-2022928 HS-GAG biosynthesis
R-HSA-2024096 HS-GAG degradation
R-HSA-3560783 Defective B4GALT7 causes EDS, progeroid type
R-HSA-3560801 Defective B3GAT3 causes JDSSDHD
R-HSA-3656237 Defective EXT2 causes exostoses 2
R-HSA-3656253 Defective EXT1 causes exostoses 1, TRPS2 and CHDS
R-HSA-381426 Regulation of Insulin-like Growth Factor (IGF) transport and uptake by Insulin-like Growth Factor Binding Proteins (IGFBPs)
R-HSA-4420332 Defective B3GALT6 causes EDSP2 and SEMDJL1
R-HSA-8957275 Post-translational protein phosphorylation
R-HSA-975634 Retinoid metabolism and transport
SignaLinkiP51654
SIGNORiP51654

Names & Taxonomyi

Protein namesi
Recommended name:
Glypican-3
Alternative name(s):
GTR2-2
Intestinal protein OCI-5
MXR7
Cleaved into the following chain:
Gene namesi
Name:GPC3
Synonyms:OCI5
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome X

Organism-specific databases

EuPathDBiHostDB:ENSG00000147257.13
HGNCiHGNC:4451 GPC3
MIMi300037 gene
neXtProtiNX_P51654

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Cell membrane, Membrane, Secreted

Pathology & Biotechi

Involvement in diseasei

Simpson-Golabi-Behmel syndrome 1 (SGBS1)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA 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.
See also OMIM:312870
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_021385296W → R in SGBS1. 1 PublicationCorresponds to variant dbSNP:rs104894854EnsemblClinVar.1

Keywords - Diseasei

Disease mutation

Organism-specific databases

DisGeNETi2719
GeneReviewsiGPC3
MalaCardsiGPC3
MIMi312870 phenotype
OpenTargetsiENSG00000147257
Orphaneti373 Simpson-Golabi-Behmel syndrome
PharmGKBiPA28832

Polymorphism and mutation databases

BioMutaiGPC3
DMDMi1708022

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
PropeptideiPRO_0000012310? – 580Removed in mature formSequence analysis
Signal peptidei1 – 24Sequence analysisAdd BLAST24
ChainiPRO_000001230925 – ?Glypican-3
ChainiPRO_000033384425 – ?Secreted glypican-3

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Glycosylationi124N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi241N-linked (GlcNAc...) asparagineSequence analysis1
Modified residuei352Phosphoserine; by FAM20C1 Publication1
Glycosylationi418N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi495O-linked (Xyl...) (heparan sulfate) serineSequence analysis1
Glycosylationi509O-linked (Xyl...) (heparan sulfate) serineSequence analysis1

Keywords - PTMi

Glycoprotein, GPI-anchor, Heparan sulfate, Lipoprotein, Phosphoprotein, Proteoglycan

Proteomic databases

MaxQBiP51654
PaxDbiP51654
PeptideAtlasiP51654
PRIDEiP51654
ProteomicsDBi56359
TopDownProteomicsiP51654-1 [P51654-1]

PTM databases

iPTMnetiP51654
PhosphoSitePlusiP51654

Expressioni

Tissue specificityi

Highly expressed in lung, liver and kidney.

Gene expression databases

BgeeiENSG00000147257
CleanExiHS_GPC3
ExpressionAtlasiP51654 baseline and differential
GenevisibleiP51654 HS

Organism-specific databases

HPAiHPA006316

Interactioni

Subunit structurei

Interacts with DPP4.1 Publication

Protein-protein interaction databases

BioGridi108983, 24 interactors
DIPiDIP-61509N
IntActiP51654, 5 interactors
STRINGi9606.ENSP00000377836

Structurei

3D structure databases

ProteinModelPortaliP51654
SMRiP51654
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the glypican family.Curated

Keywords - Domaini

Signal

Phylogenomic databases

eggNOGiKOG3821 Eukaryota
ENOG410XST2 LUCA
GeneTreeiENSGT00550000074430
HOGENOMiHOG000049177
HOVERGENiHBG005896
InParanoidiP51654
KOiK08109
OMAiMNPGLPE
OrthoDBiEOG091G06T6
PhylomeDBiP51654
TreeFamiTF105317

Family and domain databases

InterProiView protein in InterPro
IPR001863 Glypican
IPR015501 Glypican-3
IPR019803 Glypican_CS
PANTHERiPTHR10822 PTHR10822, 1 hit
PTHR10822:SF4 PTHR10822:SF4, 1 hit
PfamiView protein in Pfam
PF01153 Glypican, 1 hit
PROSITEiView protein in PROSITE
PS01207 GLYPICAN, 1 hit

Sequences (3)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 3 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: P51654-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MAGTVRTACL VVAMLLSLDF PGQAQPPPPP PDATCHQVRS FFQRLQPGLK
60 70 80 90 100
WVPETPVPGS DLQVCLPKGP TCCSRKMEEK YQLTARLNME QLLQSASMEL
110 120 130 140 150
KFLIIQNAAV FQEAFEIVVR HAKNYTNAMF KNNYPSLTPQ AFEFVGEFFT
160 170 180 190 200
DVSLYILGSD INVDDMVNEL FDSLFPVIYT QLMNPGLPDS ALDINECLRG
210 220 230 240 250
ARRDLKVFGN FPKLIMTQVS KSLQVTRIFL QALNLGIEVI NTTDHLKFSK
260 270 280 290 300
DCGRMLTRMW YCSYCQGLMM VKPCGGYCNV VMQGCMAGVV EIDKYWREYI
310 320 330 340 350
LSLEELVNGM YRIYDMENVL LGLFSTIHDS IQYVQKNAGK LTTTIGKLCA
360 370 380 390 400
HSQQRQYRSA YYPEDLFIDK KVLKVAHVEH EETLSSRRRE LIQKLKSFIS
410 420 430 440 450
FYSALPGYIC SHSPVAENDT LCWNGQELVE RYSQKAARNG MKNQFNLHEL
460 470 480 490 500
KMKGPEPVVS QIIDKLKHIN QLLRTMSMPK GRVLDKNLDE EGFESGDCGD
510 520 530 540 550
DEDECIGGSG DGMIKVKNQL RFLAELAYDL DVDDAPGNSQ QATPKDNEIS
560 570 580
TFHNLGNVHS PLKLLTSMAI SVVCFFFLVH
Length:580
Mass (Da):65,563
Last modified:October 1, 1996 - v1
Checksum:i19485B76D3CE15FC
GO
Isoform 2 (identifier: P51654-2) [UniParc]FASTAAdd to basket
Also known as: Variant B

The sequence of this isoform differs from the canonical sequence as follows:
     59-112: Missing.

Show »
Length:526
Mass (Da):59,475
Checksum:iA0858770AF739C17
GO
Isoform 3 (identifier: P51654-3) [UniParc]FASTAAdd to basket
Also known as: Variant C

The sequence of this isoform differs from the canonical sequence as follows:
     344-344: T → TETEKKIWHFKYPIFFLCIGLDLQ

Show »
Length:603
Mass (Da):68,414
Checksum:i1930C0382E91AF60
GO

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_021385296W → R in SGBS1. 1 PublicationCorresponds to variant dbSNP:rs104894854EnsemblClinVar.1
Natural variantiVAR_069139429V → M1 PublicationCorresponds to variant dbSNP:rs11539789EnsemblClinVar.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_04611759 – 112Missing in isoform 2. 1 PublicationAdd BLAST54
Alternative sequenceiVSP_046703344T → TETEKKIWHFKYPIFFLCIG LDLQ in isoform 3. 1 Publication1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U50410 mRNA Translation: AAA93471.1
L47124 Genomic DNA Translation: AAA98131.1
L47125 mRNA Translation: AAA98132.1
L47176 mRNA Translation: AAB58806.1
Z37987 mRNA Translation: CAA86069.1
DQ349136 mRNA Translation: ABC72125.1
DQ349138 mRNA Translation: ABC72127.1
AL008712
, AC002420, AF003529, AL009174, Z99570 Genomic DNA Translation: CAI43110.1
AL009174
, AC002420, AF003529, AL008712, Z99570 Genomic DNA Translation: CAI42761.1
Z99570
, AC002420, AF003529, AL008712, AL009174 Genomic DNA Translation: CAI42277.1
AL034401 Genomic DNA No translation available.
AL662851 Genomic DNA No translation available.
Z97196 Genomic DNA No translation available.
CH471107 Genomic DNA Translation: EAX11771.1
AF003529 Genomic DNA Translation: AAB87062.1
CCDSiCCDS14638.1 [P51654-1]
CCDS55495.1 [P51654-2]
CCDS55496.1 [P51654-3]
RefSeqiNP_001158089.1, NM_001164617.1 [P51654-3]
NP_001158091.1, NM_001164619.1 [P51654-2]
NP_004475.1, NM_004484.3 [P51654-1]
UniGeneiHs.644108

Genome annotation databases

EnsembliENST00000370818; ENSP00000359854; ENSG00000147257 [P51654-1]
ENST00000394299; ENSP00000377836; ENSG00000147257 [P51654-3]
ENST00000631057; ENSP00000486325; ENSG00000147257 [P51654-2]
GeneIDi2719
KEGGihsa:2719
UCSCiuc004exe.4 human [P51654-1]

Keywords - Coding sequence diversityi

Alternative splicing

Similar proteinsi

Entry informationi

Entry nameiGPC3_HUMAN
AccessioniPrimary (citable) accession number: P51654
Secondary accession number(s): C9JLE3
, G3V1R0, Q2L880, Q2L882
Entry historyiIntegrated into UniProtKB/Swiss-Prot: October 1, 1996
Last sequence update: October 1, 1996
Last modified: July 18, 2018
This is version 170 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Human chromosome X
    Human chromosome X: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. SIMILARITY comments
    Index of protein domains and families

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