Cancer Protein Description

This report provides a detailed description of a selected cancer protein with information collected from various sources, including UniProt, the Wellcome Trust Sanger Institute’s Catalogue of Somatic Mutations in Cancer (COSMIC), and the Atlas of Genetics and Cytogenetics in Oncology and Haematology.


Protein Name: HSPG2
Gene Name: HSPG2
Protein Full Name: Basement membrane-specific heparan sulfate proteog
Alias: Heparan sulfate proteoglycan 2; HSPG; Perlecan; Perlecan proteoglycan; PGBM; PLC; PRCAN; SJS1
Mass (Da): 468798
Number AA: 4391
UniProt ID: P98160
Locus ID: 3339
COSMIC ID: HSPG2
Gene location on chromosome: 1p36.12
Cancer protein type: OP/TSP
Effect of cancer mutation on protein: UNCLEAR
Effect of active protein on cancer: MIXED
Number of cancer specimens: 19675
Percent of cancer specimens with mutations: 2.7
General distribution of mutations: Multi-site
Location of most mutations: Broad distribution of mutation sites with several point mutations, but no complex, insertion or deletion mutants.
Normal role description: The protein encoded for by HSPG2 functions as an integral component of the glomerular basement membrane, where it is responsible for the fixed negative electrostatic membrane charge, which provides a barrier that is both size- and charge-selective. It also serves as an attachment substrate for cells and plays an important role in vascularization, normal heart development, and in the regulation of the vascular response to injury. Endorepellin (1 of the 2 chains HSPG2 is cleaved into - the other one being LG3 ligand) is an anti-angiogenic and anti-tumour peptide that inhibits endothelial cell migration, collagen-induced endothelial tube morphogenesis and angiogenesis in the chorioallantoic membrane. It reduces phosphorylation of RTK's by activating PTPN6. The LG3 peptide also has anti-angiogenic properties. However, HSPG2 as a whole molecule has been found to be a pro-angionetic factor and to be upregulated in a number of cancers. Defects in HSPG2 are also the cause of Schwartz-Jampel syndrome and dyssegmental dysplasia Silverman-Handmaker type.
Commentary on involvement of protein in cancer: 1/1 pharyngeal tumour sample was also mutated (mis-sense), and so were 1/44 CNS samples


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