Human heparan sulfate proteoglycan 2 (HSPG2) ELISA kit

Integral component of basement membranes. Component of the glomerular basement membrane (GBM), responsible for the fixed negative electrostatic membrane charge, and which provides a barrier which is both size- and charge-selective. It serves as an attachment substrate for cells. Plays essential roles in vascularization. Critical for normal heart development and for regulating the vascular response to injury. Also required for avascular cartilage development.; Endorepellin in 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. Blocks endothelial cell adhesion to fibronectin and type I collagen. Anti-tumor agent in neovascularization. Interaction with its ligand, integrin alpha2/beta1, is required for the anti-angiogenic properties. Evokes a reduction in phosphorylation of receptor tyrosine kinases via alpha2/beta1 integrin-mediated activation of the tyrosine phosphatase, PTPN6.; The LG3 peptide has anti-angiogenic properties that require binding of calcium ions for full activity.

1. Molecular analysis results revealed a novel homozygous variant in the HSPG2 gene (MIM 142461), NM_005529.6(HSPG2):c.4029 + 1G>A, consistent with a diagnosis of Dyssegmental dysplasia, Silverman-Handmaker type. PMID: 29526034
2. The results of the present study suggested that the compound heterozygous mutations in HSPG2 may be responsible the induction of Schwartz-Jampel syndrome type 1 (SJS1), and demonstrated the genotypephenotype associations between mutations in the HSPG2 gene and clinical characteristics of SJS1 PMID: 29901129
3. The differential immunoexpression of perlecan and biglycan in these types of ameloblastomas suggests their participation in the developmental process of these tumors. PMID: 29921372
4. The Mechanistic studies showed that CSPG4 bound to perlecan via hydrophobic protein-protein interactions involving multiple sites on perlecan including the C-terminal region. PMID: 29462330
5. The results indicate that increase of heparan sulfate content and up-regulation of perlecan/HSPG2 expression in glioblastoma tissues contribute to tumour development through the transformation of brain extracellular matrix into tumour microenvironment, and represent negative prognostic factors for glioblastoma progression. PMID: 29322326
6. Mutations in this gene are responsible for the allelic Skeletal Dysplasias Schwartz-Jampel syndrome type 1 and the Silverman-Handmaker type of Dyssegmental Dysplasia, both of which are autosomal recessive. PMID: 28570402
7. Perlecan functions in autophagy and angiogenesis where its proangiogenesis activity is counteracted by endorepellin, the C-terminal fragment of perlecan, in these cellular and morphogenic events. (Review) PMID: 27613501
8. We found that perlecan expression decreased during chronological skin aging. Our in vitro studies revealed reduced perlecan transcript levels in aged keratinocytes. Perlecan down-regulation in cultured keratinocytes caused depletion of the cell population that expressed keratin 15.Finally, we found defects in keratin 15 expression in the epidermis of aging skin. PMID: 26996820
9. Putative stem cell populations associated with hair bulbs, humeral perichondrium, humeral and ulnar rudiment stromal/perivascular tissues expressed the Chondroitin sulfate motifs 4C3, 7D4, and 3B3[-] along with perlecan in close association but not colocalized. PMID: 27068010
10. autophagy is a novel mechanism by which endorepellin promotes angiostasis independent of nutrient deprivation. PMID: 27435676
11. Heterozygous variants in HSPG2 regulate the ATP2B4 expression via a variety of transcription factors including GATA1, RFX1 and MAZ. PMID: 28327142
12. the HSPG2-rs3767140 might be associated with the decreased fasting plasma glucose and LDL-C and with the increased HDL-C in diabetics. PMID: 27545212
13. Together, perlecan fragments in sera and MMP-7 in tissues of Prostate cancer patients are measures of invasive Prostate cancer. PMID: 26862737
14. Results show that perlecan has physical properties that would allow it to act as a strong but elastic tether in the lacunar canalicular system of cortical bone. PMID: 26546708
15. We were able to identify perlecan as the most likely candidate for the major estrogen-binding protein in the follicular fluid. PMID: 26552664
16. Knockdown of agrin and perlecan promoted a decrease on cell migration and adhesion, and on resistance of cells to cisplatin. PMID: 25506919
17. As five of the seven missense mutations in Schwartz-Jampel syndrome affect domain III of perlecan, domain III is likely to be essential for secretion of perlecan into the extracellular space. PMID: 26031903
18. Rare variants in the HSPG2 gene potentially contribute to the idiopathic scoliosis phenotype in a subset of patients with idiopathic scoliosis PMID: 25504735
19. The perlecan is the primary ECM molecule comprising intraepithelial stroma of the junctional epithelium, in which leukocytes may migrate on ECM scaffolds in intercellular space toward the surface of the gingival sulci or pockets. PMID: 24562868
20. We conclude that enzymatic processing of perlecan in the BM or territorial matrix by MMP-7 as occurs in the invasive tumor microenvironment acts as a molecular switch to alter PCa cell behavior and favor cell dispersion and invasiveness. PMID: 24833109
21. Mutant genes (CELA1, HSPG2, and KCNK5) in Balkan endemic nephropathy patients encode proteins involved in basement membrane/extracellular matrix and vascular tone, tightly connected to process of angiogenesis. PMID: 24949484
22. this study hypothesizes the transcriptional control of the HSPG2 gene in mast cells to synthesize these transcripts supports their stimulatory and specific role in wound healing and tissue regeneration. PMID: 24365408
23. Perlecan synthesized by smooth muscle cells differs from that synthesized by endothelial cells by possessing different signaling capabilities and thus differential modulation of cell adhesion, proliferation and growth factor signaling. PMID: 24509440
24. role for perlecan in chondrogenic and osteogenic events which drive discal development and ossification of the vertebral bodies. PMID: 23397188
25. endorepellin glycoforms may be highly specific and sensitive biomarkers for the differentiation of mucinous from nonmucinous pancreatic cysts. PMID: 23836919
26. Urinary perlecan laminin G-like 3 peptide and Ig kappa light chains were decreased in IgA nephropathy. PMID: 23599406
27. Data suggest that cancer cell-derived exosomes use heparan sulfate proteoglycans (HSPGs) for their internalization and functional activity, which significantly extends the emerging role of HSPGs as key receptors of macromolecular cargo. PMID: 24101524
28. [review] Perlecan domain V reached the infarcted brain tissue and peri-infarct brain regions because a transient middle cerebral artery occlusion model allowed for vascular reperfusion to the stroked brain region after 1 h of experimental occlusion. PMID: 23509972
29. No association has been found between polymorphisms of rs251124 and rs3767137 loci of CSPG2 and HSPG2 genes and intracranial aneurysm in the selected population. PMID: 23568740
30. Endorepellin binds through its proximal LG1/2 domains to VEGFR2 and inhibit VEGFA-dependent endothelial migration. PMID: 23374253
31. we suggest that the LG3 fragment of endorepellin could be associated with IgA nephropathy severity and might be related to pathogenesis of IgA nephropathy . PMID: 23161552
32. Based on genetic analysis of patients with BA and zebrafish, GPC1 appears to be a BA susceptibility gene. These findings also support a role for Hedgehog signaling in the pathogenesis of BA PMID: 23336978
33. The overexpression of hypomethylated miR-663 induces chemotherapy resistance in human breast cancer cells by targeting heparin sulfate proteoglycan 2 (HSPG2). PMID: 23436656
34. Report immunolocalization of fibrillin-1/perlecan in human fetal intervertebral disc. PMID: 23104139
35. Domain V of perlecan, a known alpha2 integrin ligand, inhibits brain amyloid-beta neurotoxicity in an alpha2 integrin-dependent manner. PMID: 21126803
36. association of the HSPG2 intronic SNP, rs2445142, with tardive dyskinesia susceptibility was demonstrated. PMID: 21808285
37. The C-terminal fragment of the extracellular matrix component perlecan (domain V, DV) has been shown to be increased in arteriovenous malformation of the brain. PMID: 22643235
38. This study shows for the first time that mast cells secrete and process the extracellular proteoglycan perlecan into fragments containing the endorepellin C-terminal region that regulate angiogenesis and matrix turnover. PMID: 23235151
39. activity mediated release of LG3/endorepellin into the circulation may represent a biological mechanism for the known inverse association between physical activity and cancer risk/survival PMID: 22457785
40. The perlecan fragment LG3 is a novel regulator of obliterative remodeling associated with allograft vascular rejection. PMID: 22076637
41. TGF-beta(1)-induced perlecan deposition may enhance attachment of migrating airway smooth muscle cells (ASMC) in vivo and thus may be a mechanism for ASMC layer hypertrophy in chronic obstructive pulmonary disease PMID: 22003087
42. endorepellin requires both the alpha2beta1 integrin and VEGFR2 for its angiostatic activity PMID: 21596751
43. Ameloblastoma cells proliferate and are differentiated by capturing perlecan differentially with alpha-dystroglycan and integrin beta1, respectively PMID: 21255062
44. The expression level of perlecan and perlecan mRNA significantly increased in Hep-2 cells as compared with normal cells. PMID: 16570819
45. perlecan followed virtually identical immunolocalisation pattern to type II collagen in foetal joint tissue, but a slightly divergent pattern in adult tissues; evidence indicates perlecan is a marker of chondrogenic cells in prenatal cartilages PMID: 20690028
46. FGF2 and -18 bind to discrete structures on the heparan sulfate chains attached to chondrocyte-derived perlecan which modulate the growth factor activities PMID: 20507176
47. in contrast to IA, HSPG2 and CSPG2 do not associate with AAA. PMID: 20053631
48. These findings suggest that the HSPG2 gene is involved in neuroleptic-induced tardive dyskinesia (TD) and higher expression of HSPG2, probably even after antipsychotic treatment, and may be associated with TD susceptibility. PMID: 20072119
49. These data highlight the potential role of perlecan oxidation, and consequent deregulation of cell function, in vascular injuries by myeloperoxidase-derived hypochlorous and hypobromous acids. PMID: 19788922
50. perlecan plays an indispensible role in endothelial cell proliferation and acts through a mechanism that involves subcellular localization of p27. PMID: 20074558

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Schwartz-Jampel syndrome (SJS1); Dyssegmental dysplasia Silverman-Handmaker type (DDSH)

Secreted, extracellular space, extracellular matrix, basement membrane.

Found in the basement membranes.

HGNC: 5273

OMIM: 142461

KEGG: hsa:3339

STRING: 9606.ENSP00000363827

UniGene: Hs.562227