HSPG2
HSPG2 (heparan sulfate proteoglycan 2), also known as perlecan, PRCAN and SJS1, is a large multidomain proteoglycan of the extracellular matrix. It is a major component of basement membranes and binds and cross-links many matrix components and cell-surface molecules. Browse Assay Genie's current range of HSPG2 research reagents below, or read on for the biology behind the target.
HSPG2 encodes perlecan, a core protein to which three long chains of glycosaminoglycans, either heparan sulfate or chondroitin sulfate, are attached. This large multidomain proteoglycan binds to and cross-links many extracellular matrix components and cell-surface molecules. Reported interaction partners include laminin, prolargin, collagen type IV, FGFBP1, FBLN2, FGF7 and transthyretin. In basement membranes, perlecan helps stabilise other molecules and contributes to cell adhesion and to glomerular permeability to macromolecules. The protein is secreted into the extracellular space and basement membrane.
Perlecan is a key component of the vascular extracellular matrix, where it helps to maintain endothelial barrier function. It is a potent inhibitor of smooth muscle cell proliferation and is therefore thought to help maintain vascular homeostasis. It can also promote the activity of growth factors such as FGF2, and so stimulate endothelial growth and regeneration. UniProt additionally annotates anti-angiogenic properties that require calcium ion binding for full activity. These roles make the HSPG2 protein relevant to studies of vascular biology, matrix organisation and growth factor presentation.
The HSPG2 gene is placed by HGNC in the proteoglycan, I-set domain containing and SEA domain containing groups. Alternative splicing produces multiple transcript variants. Mutations in the gene cause Schwartz-Jampel syndrome type 1, the origin of the earlier name Schwartz-Jampel syndrome 1 (chondrodystrophic myotonia) and the SJS1 alias. Mutations are also linked to the Silverman-Handmaker type of dyssegmental dysplasia and to tardive dyskinesia. The mouse ortholog is Hspg2.
Perlecan is a major basement membrane component that stabilises other molecules and supports cell adhesion and glomerular permeability to macromolecules.
Perlecan helps maintain endothelial barrier function and inhibits smooth muscle cell proliferation in the vascular extracellular matrix.
Perlecan can promote FGF2 activity and endothelial regeneration, and carries calcium-dependent anti-angiogenic properties.
Mutations in HSPG2 cause Schwartz-Jampel syndrome type 1 and Silverman-Handmaker dyssegmental dysplasia, and are linked to tardive dyskinesia.
HSPG2 encodes perlecan, a proteoglycan that binds and cross-links extracellular matrix components and cell-surface molecules. It is a major basement membrane component, supports cell adhesion and glomerular permeability, helps maintain endothelial barrier function, and can promote FGF2 activity. Check the datasheet of any product for its validated reactivity.
HSPG2 is widely called perlecan, and is also known as PRCAN and SJS1. The SJS1 alias and the earlier name Schwartz-Jampel syndrome 1 (chondrodystrophic myotonia) reflect the disorder caused by mutations in the gene. UniProt names the protein basement membrane-specific heparan sulfate proteoglycan core protein.
Yes. According to the NCBI Gene summary, mutations in HSPG2 cause Schwartz-Jampel syndrome type 1 and the Silverman-Handmaker type of dyssegmental dysplasia, and are associated with tardive dyskinesia. The gene has a mouse ortholog, Hspg2, and undergoes alternative splicing to give multiple transcript variants.
Growth factors, basement membrane proteins and extracellular matrix partners that interact with perlecan.
Gene and protein annotations from HGNC, NCBI Gene and UniProt P98160; product specifications from Assay Genie datasheets.