Aliases

• Endorepellin (Domain V Region)
• HSPG2
• PLC
• PRCAN
• SJS
• SJS1
• Schwartz-Jampel syndrome 1 (chondrodystrophic myotonia)
• endorepellin
• heparan sulfate proteoglycan 2
• perlecan
• perlecan proteoglycan

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

HGNC Name

HSPG2