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HSPG2

Basics

Aliases:
This biomarker is also known as:
  • PLC,
  • SJS1,
  • perlecan proteoglycan,
  • SJS,
  • Schwartz-Jampel syndrome 1 (chondrodystrophic myotonia),
  • heparan sulfate proteoglycan 2,
  • perlecan,
  • PRCAN,

View in BioMuta

Description…

HSPG2, which consists of a core protein to which three long chains of glycosaminoglycans (heparan sulfate or chondroitin sulfate) are attached, is a large multidomain proteoglycan that binds to and cross-links many extracellular matrix components and cell-surface molecules. It has been shown that HSPG2 interacts with other basement membrane components (such as laminin, prolargin, collagen type IV, FGFBP1, FBLN2, FGF7 and Transthyretin). HSPG2 is a key component of the vascular extracellular matrix, where it helps to maintain the endothelial barrier function; it is responsible for the fixed negative electrostatic membrane charge, and which provides a barrier which is both size- and charge-selective. 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. HSPG2 is cleaved into two chains, Endorepellin and LG3 peptide. The endorepellin chain is an anti-angiogenic and anti-tumor peptide that inhibits endothelial cell migration, collagen-induced endothelial tube morphogenesis and blood vessel growth in the chorioallantoic membrane. It blocks endothelial cell adhesion to fibronectin and type I collagen and acts as an anti-tumor agent in neovascularization.

Attributes

QA State: Under Review
Type: Protein
Short Name:
HGNC Name: HSPG2

Datasets

There are no datasets associated with this biomarker.

Organs

This biomarker is currently being annotated or is under review. You must be logged in or do not have permission to view any additional information. Contact Heather Kincaid at heather.kincaid@jpl.nasa.gov if you should have access to this biomarker.

Studies

This biomarker is currently being annotated or is under review. You must be logged in or do not have permission to view any additional information. Contact Heather Kincaid at heather.kincaid@jpl.nasa.gov if you should have access to this biomarker.

Publications

This biomarker is currently being annotated or is under review. You must be logged in or do not have permission to view any additional information. Contact Heather Kincaid at heather.kincaid@jpl.nasa.gov if you should have access to this biomarker.

Resources

This biomarker is currently being annotated or is under review. You must be logged in or do not have permission to view any additional information. Contact Heather Kincaid at heather.kincaid@jpl.nasa.gov if you should have access to this biomarker.

Announcement 10/07/2014

EDRN Patient Advocates will host an EDRN Advocacy Educational Webinar, Biomarkers for Prostate Cancer Detection and Monitoring, on Monday, January 12th, 2015, at 1 p.m. EDT / 10 a.m. PDT. Registration is not required for this. Please click for more information.
Announcement 09/14/2014

Thank you to everyone who helped make the 9th EDRN Scientific Workshop a success. We look forward to seeing everyone at the 28th EDRN Steering Committee Meeting from March 31-April 2, 2015, in Atlanta, GA.