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Details on Person UniProt:P98160 HSPG2

Class:IdReferenceGeneProduct:61584
_chainChangeLogsignal peptide:1-21 added on Fri February 6 2015;chain:22-4391 added on Fri February 6 2015;chain:3687-4391 added on Fri February 6 2015;chain:4197-4391 added on Fri February 6 2015
_displayNameUniProt:P98160 HSPG2
_timestamp2026-02-20 22:02:34
chainsignal peptide:1-21
chain:22-4391
chain:3687-4391
chain:4197-4391
checksumC587660E24C83324
commentFUNCTION Integral component of basement membranes. Component of the glomerular basement membrane (GBM), responsible for the fixed negative electrostatic membrane charge, and which provides a barrier which is both size- and charge-selective. It serves as an attachment substrate for cells. Plays essential roles in vascularization. Critical for normal heart development and for regulating the vascular response to injury. Also required for avascular cartilage development (PubMed:12435733, PubMed:15591058, PubMed:19789387). In muscle, it is essential for localizing acetylcholinesterase (AChE) at the neuromuscular junctions (NMJ), most probably acting as an adapter that links the acetylcholinesterase collagenic tail peptide (COLQ) to alpha-dystroglycan, and is therefore involved in the down-regulation of colinergic synaptic transmission (By similarity).FUNCTION Anti-angiogenic and anti-tumor peptide that inhibits endothelial cell migration, collagen-induced endothelial tube morphogenesis and blood vessel growth in the chorioallantoic membrane. Blocks endothelial cell adhesion to fibronectin and type I collagen. Anti-tumor agent in neovascularization. Interaction with its ligand, integrin alpha2/beta1, is required for the anti-angiogenic properties. Evokes a reduction in phosphorylation of receptor tyrosine kinases via alpha2/beta1 integrin-mediated activation of the tyrosine phosphatase, PTPN6.FUNCTION Has anti-angiogenic properties that require binding of calcium ions for full activity.SUBUNIT Has a strong tendency to aggregate in dimers or stellate structures. Interacts with other basement membrane components such as laminin, prolargin and collagen type IV. Interacts with COL13A1 (PubMed:11956183). Interacts with FGFBP1 (PubMed:11148217). Interacts with VWA1 (By similarity). Interacts (via C-terminus) with ECM1 (via C-terminus) (PubMed:12604605). Interacts with SVEP1 (By similarity). Interacts (via C-terminus) with alpha-dystroglycan; the interaction is required for acetylcholinesterase (AChE) localization at the neuromuscular junctions (NMJ) (By similarity). Interacts with the acetylcholinesterase collagenic tail peptide (COLQ) (PubMed:14702351).INTERACTION Detected in cerebrospinal fluid, fibroblasts and urine (at protein level).PTM Proteolytic processing produces the C-terminal angiogenic peptide, endorepellin. This peptide can be further processed to produce the LG3 peptide.PTM O-glycosylated with core 1 or possibly core 8 glycans. Contains three heparan sulfate chains. Also contains chondroitin sulfate.DISEASE The disease is caused by variants affecting the gene represented in this entry.DISEASE The disease is caused by variants affecting the gene represented in this entry.MISCELLANEOUS Has been found in the urine of patients with end-stage renal disease and in the amniotic fluid of pregnant women with premature rupture of fetal membranes.ONLINE INFORMATION Perlecan entry
descriptionrecommendedName: Basement membrane-specific heparan sulfate proteoglycan core protein shortName: HSPG alternativeName: Perlecan shortName: PLC component recommendedName: Endorepellin /component component recommendedName: LG3 peptide /component
geneNameHSPG2
identifierP98160
isSequenceChangedFALSE
keyword3D-structure
Angiogenesis
Basement membrane
Calcium
Direct protein sequencing
Disease variant
Disulfide bond
EGF-like domain
Extracellular matrix
Glycoprotein
Heparan sulfate
Immunoglobulin domain
Laminin EGF-like domain
Metal-binding
Proteoglycan
Proteomics identification
Reference proteome
Repeat
Secreted
Signal
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9862192] Weiser, Joel, 2024-02-26
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
[InstanceEdit:9983091] Weiser, Joel, 2026-02-20
nameHSPG2
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8958268] ENSEMBL:ENSG00000142798 HSPG2 [Homo sapiens]
secondaryIdentifierPGBM_HUMAN
Q16287
Q5SZI3
Q9H3V5
sequenceLength4391
species[Species:48887] Homo sapiens
(referenceEntity)[EntityWithAccessionedSequence:174719] HSPG2(22-4391) [plasma membrane] [Homo sapiens]
[EntityWithAccessionedSequence:1011570] HSPG2(22-4391) [extracellular region] [Homo sapiens]
[EntityWithAccessionedSequence:2054109] HSPG2(22-4391) [Golgi lumen] [Homo sapiens]
[EntityWithAccessionedSequence:2063971] Gal-Gal-Xyl-HSPG2 [Golgi lumen] [Homo sapiens]
[EntityWithAccessionedSequence:2063972] XylS-HSPG2(22-4391) [Golgi lumen] [Homo sapiens]
[EntityWithAccessionedSequence:2064023] GlcA-Gal-Gal-Xyl-HSPG2 [Golgi lumen] [Homo sapiens]
[EntityWithAccessionedSequence:2064209] Gal-Xyl-HSPG2 [Golgi lumen] [Homo sapiens]
[EntityWithAccessionedSequence:2076323] HS(5)-HSPG2 [Golgi lumen] [Homo sapiens]
[EntityWithAccessionedSequence:2076334] Heparan(3)-HSPG2 [Golgi lumen] [Homo sapiens]
[EntityWithAccessionedSequence:2076335] Heparan(2)-HSPG2 [Golgi lumen] [Homo sapiens]
List all 34 refering instances
(referenceSequence)[GroupModifiedResidue:2063973] O-xylosyl-L-serine (3-O-(β-D-glucuronosyl-(1→3)-β-D-galactosyl-(1→3)-β-D-galactosyl-(1→4)-β-D-xylosyl)-L-serine residue) at unknown position
[GroupModifiedResidue:2064085] O-xylosyl-L-serine (O3-(β-D-galactosyl-(1→3)-β-D-galactosyl-(1→4)-β-D-xylosyl)-L-serine residue) at unknown position
[GroupModifiedResidue:2064199] O-xylosyl-L-serine (O3-(4-β-D-galactosyl-β-D-xylosyl)-L-serine residue) at unknown position
[ModifiedResidue:2064239] O-xylosyl-L-serine at unknown position
[GroupModifiedResidue:2076333] O-xylosyl-L-serine (ChEBI:63645) at unknown position
[GroupModifiedResidue:2076436] O-xylosyl-L-serine (ChEBI:63649) at unknown position
[GroupModifiedResidue:2076445] O-xylosyl-L-serine (ChEBI:63654) at unknown position
[GroupModifiedResidue:2076463] O-xylosyl-L-serine (ChEBI:63651) at unknown position
[GroupModifiedResidue:2076484] O-xylosyl-L-serine (ChEBI:63657) at unknown position
[GroupModifiedResidue:2076519] O-xylosyl-L-serine (ChEBI:63646) at unknown position
List all 17 refering instances
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No pathways have been reviewed or authored by UniProt:P98160 HSPG2 (61584)