Heparan Sulfate Proteoglycan 2 (HSPG2)

PLC; PRCAN; SJA; SJS; SJS1; Perlecan; Endorepellin; Schwartz-Jampel Syndrome 1; Basement membrane-specific heparan sulfate proteoglycan core protein

SPN	U109
HGNC	5273
MGI	96257
PubMed	16927315
UniProt	P98160
Wiki	Perlecan

Perlecan is a large multidomain proteoglycan that binds to and cross-links many extracellular matrix (ECM) components and cell-surface molecules.Perlecan is synthesized by both vascular endothelial and smooth muscle cells and deposited in the extracellular matrix. Perlecan is highly conserved across species and the available data indicate that it has evolved from ancient ancestors by gene duplication and exon shuffling.Perlecan is a key component of the vascular extracellular matrix, here it interacts with a variety of other matrix components and helps to maintain the endothelial barrier function. Perlecan is a potent inhibitor of smooth muscle cell proliferation and is thus thought to help maintain vascular homeostasis. Perlecan can also promote growth factor activity and thus stimulate endothelial growth and re-generation.

Reference

"Primary structure of the human heparan sulfate proteoglycan from basement membrane (HSPG2/perlecan). A chimeric molecule with multiple domains homologous to the low density lipoprotein receptor, laminin, neural cell adhesion molecules, and epidermal growth factor."
J. Biol. Chem. 267:8544-8557(1992) [PubMed] [Europe PMC] [Abstract]

"Human basement membrane heparan sulfate proteoglycan core protein: a 467-kD protein containing multiple domains resembling elements of the low density lipoprotein receptor, laminin, neural cell adhesion molecules, and epidermal growth factor."
J. Cell Biol. 116:559-571(1992) [PubMed] [Europe PMC] [Abstract]

"The DNA sequence and biological annotation of human chromosome 1."
Nature 441:315-321(2006) [PubMed] [Europe PMC] [Abstract]

"Structural characterization of the complete human perlecan gene and its promoter."
Proc. Natl. Acad. Sci. U.S.A. 90:10404-10408(1993) [PubMed] [Europe PMC] [Abstract]

"Perlecan, the major proteoglycan of basement membranes, is altered in patients with Schwartz-Jampel syndrome (chondrodystrophic myotonia)."
Nat. Genet. 26:480-483(2000) [PubMed] [Europe PMC] [Abstract]

"Cloning of human heparan sulfate proteoglycan core protein, assignment of the gene (HSPG2) to 1p36.1-->p35 and identification of a BamHI restriction fragment length polymorphism."
Genomics 11:389-396(1991) [PubMed] [Europe PMC] [Abstract]

"Heparan sulfate proteoglycan of human colon: partial molecular cloning, cellular expression, and mapping of the gene (HSPG2) to the short arm of human chromosome 1."
Genomics 10:673-680(1991) [PubMed] [Europe PMC] [Abstract]

"Matrix-associated heparan sulfate proteoglycan: core protein-specific monoclonal antibodies decorate the pericellular matrix of connective tissue cells and the stromal side of basement membranes."
J. Cell Biol. 109:3199-3211(1989) [PubMed] [Europe PMC] [Abstract]

"BMP-1/Tolloid-like metalloproteases process endorepellin, the angiostatic C-terminal fragment of perlecan."
J. Biol. Chem. 280:7080-7087(2005) [PubMed] [Europe PMC] [Abstract]

"Endorepellin, a novel inhibitor of angiogenesis derived from the C terminus of perlecan."
J. Biol. Chem. 278:4238-4249(2003) [PubMed] [Europe PMC] [Abstract]

"Fibroblast growth factor-binding protein is a novel partner for perlecan protein core."
J. Biol. Chem. 276:10263-10271(2001) [PubMed] [Europe PMC] [Abstract]

"Dyssegmental dysplasia, Silverman-Handmaker type, is caused by functional null mutations of the perlecan gene."
Nat. Genet. 27:431-434(2001) [PubMed] [Europe PMC] [Abstract]

"The type XIII collagen ectodomain is a 150-nm rod and capable of binding to fibronectin, nidogen-2, perlecan, and heparin."
J. Biol. Chem. 277:23092-23099(2002) [PubMed] [Europe PMC] [Abstract]

"Perlecan protein core interacts with extracellular matrix protein 1 (ECM1), a glycoprotein involved in bone formation and angiogenesis."
J. Biol. Chem. 278:17491-17499(2003) [PubMed] [Europe PMC] [Abstract]

"Identification and quantification of N-linked glycoproteins using hydrazide chemistry, stable isotope labeling and mass spectrometry."
Nat. Biotechnol. 21:660-666(2003) [PubMed] [Europe PMC] [Abstract]

"Human plasma N-glycoproteome analysis by immunoaffinity subtraction, hydrazide chemistry, and mass spectrometry."
J. Proteome Res. 4:2070-2080(2005) [PubMed] [Europe PMC] [Abstract]

"Endorepellin in vivo: targeting the tumor vasculature and retarding cancer growth and metabolism."
J. Natl. Cancer Inst. 98:1634-1646(2006) [PubMed] [Europe PMC] [Abstract]

"Integrin alpha2beta1 is the required receptor for endorepellin angiostatic activity."
J. Biol. Chem. 283:2335-2343(2008) [PubMed] [Europe PMC] [Abstract]

"Role of tyrosine phosphatase SHP-1 in the mechanism of endorepellin angiostatic activity."
Blood 114:4897-4906(2009) [PubMed] [Europe PMC] [Abstract]

"Glycoproteomics analysis of human liver tissue by combination of multiple enzyme digestion and hydrazide chemistry."
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"Human urinary glycoproteomics; attachment site specific analysis of N-and O-linked glycosylations by CID and ECD."
Mol. Cell. Proteomics 0:0-0(2011) [PubMed] [Europe PMC] [Abstract]

"An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."
J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]

"Crystal structure of the LG3 domain of endorepellin, an angiogenesis inhibitor."
J. Mol. Biol. 414:231-242(2011) [PubMed] [Europe PMC] [Abstract]