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Protein

Glypican-3

Gene

GPC3

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-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

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Protease inhibitor

Enzyme and pathway databases

BioCyciZFISH:ENSG00000147257-MONOMER.
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-4420332. Defective B3GALT6 causes EDSP2 and SEMDJL1.
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

HGNCiHGNC:4451. GPC3.

Subcellular locationi

GO - Cellular componenti

Complete GO annotation...

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 rs104894854dbSNPEnsembl.1

Keywords - Diseasei

Disease mutation

Organism-specific databases

DisGeNETi2719.
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...)Sequence analysis1
Glycosylationi241N-linked (GlcNAc...)Sequence analysis1
Modified residuei352Phosphoserine; by FAM20C1 Publication1
Glycosylationi418N-linked (GlcNAc...)Sequence analysis1
Glycosylationi495O-linked (Xyl...) (heparan sulfate)Sequence analysis1
Glycosylationi509O-linked (Xyl...) (heparan sulfate)Sequence analysis1

Keywords - PTMi

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

Proteomic databases

MaxQBiP51654.
PaxDbiP51654.
PeptideAtlasiP51654.
PRIDEiP51654.
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

HPAiCAB017784.
HPA006316.

Interactioni

Subunit structurei

Interacts with DPP4.1 Publication

Protein-protein interaction databases

BioGridi108983. 17 interactors.
DIPiDIP-61509N.
IntActiP51654. 2 interactors.
STRINGi9606.ENSP00000377836.

Structurei

3D structure databases

ProteinModelPortaliP51654.
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

InterProiIPR001863. Glypican.
IPR015501. Glypican-3.
IPR019803. Glypican_CS.
[Graphical view]
PANTHERiPTHR10822. PTHR10822. 1 hit.
PTHR10822:SF4. PTHR10822:SF4. 1 hit.
PfamiPF01153. Glypican. 1 hit.
[Graphical view]
PROSITEiPS01207. GLYPICAN. 1 hit.
[Graphical view]

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 rs104894854dbSNPEnsembl.1
Natural variantiVAR_069139429V → M.1 PublicationCorresponds to variant rs413112608dbSNPEnsembl.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

Cross-referencesi

Web resourcesi

Atlas of Genetics and Cytogenetics in Oncology and Haematology

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.

3D structure databases

ProteinModelPortaliP51654.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi108983. 17 interactors.
DIPiDIP-61509N.
IntActiP51654. 2 interactors.
STRINGi9606.ENSP00000377836.

PTM databases

iPTMnetiP51654.
PhosphoSitePlusiP51654.

Polymorphism and mutation databases

BioMutaiGPC3.
DMDMi1708022.

Proteomic databases

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

Protocols and materials databases

DNASUi2719.
Structural Biology KnowledgebaseSearch...

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]

Organism-specific databases

CTDi2719.
DisGeNETi2719.
GeneCardsiGPC3.
GeneReviewsiGPC3.
HGNCiHGNC:4451. GPC3.
HPAiCAB017784.
HPA006316.
MalaCardsiGPC3.
MIMi300037. gene.
312870. phenotype.
neXtProtiNX_P51654.
OpenTargetsiENSG00000147257.
Orphaneti373. Simpson-Golabi-Behmel syndrome.
PharmGKBiPA28832.
GenAtlasiSearch...

Phylogenomic databases

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

Enzyme and pathway databases

BioCyciZFISH:ENSG00000147257-MONOMER.
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-4420332. Defective B3GALT6 causes EDSP2 and SEMDJL1.
R-HSA-975634. Retinoid metabolism and transport.
SignaLinkiP51654.
SIGNORiP51654.

Miscellaneous databases

ChiTaRSiGPC3. human.
GeneWikiiGlypican_3.
GenomeRNAii2719.
PROiP51654.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000147257.
CleanExiHS_GPC3.
ExpressionAtlasiP51654. baseline and differential.
GenevisibleiP51654. HS.

Family and domain databases

InterProiIPR001863. Glypican.
IPR015501. Glypican-3.
IPR019803. Glypican_CS.
[Graphical view]
PANTHERiPTHR10822. PTHR10822. 1 hit.
PTHR10822:SF4. PTHR10822:SF4. 1 hit.
PfamiPF01153. Glypican. 1 hit.
[Graphical view]
PROSITEiPS01207. GLYPICAN. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiGPC3_HUMAN
AccessioniPrimary (citable) accession number: P51654
Secondary accession number(s): C9JLE3
, G3V1R0, Q2L880, Q2L882
Entry historyi
Integrated into UniProtKB/Swiss-Prot: October 1, 1996
Last sequence update: October 1, 1996
Last modified: November 30, 2016
This is version 157 of the entry and version 1 of the sequence. [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

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.