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Description for Protein HSPG2

heparan sulfate proteoglycan 2
28 total interacting proteins; 14 platelet interacting proteins
Icon Book Platelet Evidence (proteome studies/others : 0/0)
(Not detected in platelets)
Summary:
This gene encodes the perlecan protein, which consists of a core protein to which three long chains of glycosaminoglycans (heparan sulfate or chondroitin sulfate) are attached. The perlecan protein is a large multidomain proteoglycan that binds to and cross-links many extracellular matrix components and cell-surface molecules. It has been shown that this protein interacts with laminin, prolargin, collagen type IV, FGFBP1, FBLN2, FGF7 and Transthyretin, etc. and plays essential roles in multiple biological activities. Perlecan is a key component of the vascular extracellular matrix, where it helps to maintain the endothelial barrier function. It is a potent inhibitor of smooth muscle cell proliferation and is thus thought to help maintain vascular homeostasis. It can also promote growth factor (e.g., FGF2) activity and thus stimulate endothelial growth and re-generation. It is a major component of basement membranes, where it is involved in the stabilization of other molecules as well as being involved with glomerular permeability to macromolecules and cell adhesion. Mutations in this gene cause Schwartz-Jampel syndrome type 1, Silverman-Handmaker type of dyssegmental dysplasia, and Tardive dyskinesia. (PubMed Links)
Domains and Motifs:
  • SP: Signal Peptide
  • EGFLAM: Laminin-type epidermal growth factor-like domain
  • LAMB: Laminin B domain
  • IGC2: Immunoglobulin C-2 Type
  • LDLA: Low-density lipoprotein receptor domain class A
  • LAMG: Laminin G domain
  • EGF: Epidermal growth factor-like domain.
  • SEA: Domain found in sea urchin sperm protein, enterokinase, agrin
    • Gene Ontology:
    Gene Ontology Annotations
    KEGG - Enzyme ID(s):
    None Available
    KEGG - Orthology:
    K06255
    KEGG - Pathway(s):
    hsa04512
    (The yellow boxes represents platelet proteins)
    Nomenclature / Alternative Names:
    Heparan sulfate proteoglycan of basement membrane; PLC; SJA; SJS; SJS1; Heparan sulfate proteoglycan 2; Endorepellin domain V region
    Approved Symbol:
    HSPG2
    (De-) Phosphorylations:
    Total (de-) phosphorylation sites: 4
    Human (de-) phosphorylation sites: 4; No platelet phosphorylation sites
    Phosphorylation Targets:
    Total phosphorylation targets: 0
    Human phosphorylation targets: 0;Predicted platelet targets: 0
    		Protein Characteristics:
    Isoform-specific Information
    Icon DrugsAssociated Drugs (DrugBank Accession):

  • Palifermin(db)

    Associated Genetic Diseases:

  • Dyssegmental dysplasia, Silverman-Handmaker type(Pd);
  • Schwartz-Jampel syndrome, type 1(Pd)
    		• Predicted Transmembrane Domains:
  • Isoform 1 : 0
    • Additional Identifiers:

    HPRD: 00804 Entrez Gene ID: 3339 OMIM ID: 142461 Swissprot Accession: P98160;