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Rat Anti-HSPG2 Recombinant Antibody (CBFYE-0130) (CBMAB-E0256-FY)


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Summary

Host Animal
Rat
Specificity
Human, Mouse, Pig, Fish, Monkey
Clone
CBFYE-0130
Antibody Isotype
IgG2a, κ
Application
FC, IHC-P, IF

Basic Information

Immunogen
Murine EHS laminin preparation
Specificity
Human, Mouse, Pig, Fish, Monkey
Antibody Isotype
IgG2a, κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Heparan Sulfate Proteoglycan 2
Introduction
This gene encodes the perlecan protein, which consists of a core protein to which three long chains of glycosaminoglycans (heparan sulfate or chondroitin sulfate) are attached. The perlecan protein is a large multidomain proteoglycan that binds to and cross-links many extracellular matrix components and cell-surface molecules. It has been shown that this protein interacts with laminin, prolargin, collagen type IV, FGFBP1, FBLN2, FGF7 and transthyretin, etc., and it plays essential roles in multiple biological activities. Perlecan is a key component of the vascular extracellular matrix, where it helps to maintain the endothelial barrier function. 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. Alternative splicing of this gene results in multiple transcript variants.
Entrez Gene ID
Human3339
Mouse15530
Pig100517288
Zebrafish565429
Monkey707213
UniProt ID
HumanP98160
MouseQ05793
PigA0A287AF47
ZebrafishB2RZI0
MonkeyA0A2K5US37
Alternative Names
Heparan Sulfate Proteoglycan 2; Perlecan Proteoglycan; HSPG; PLC; Basement Membrane-Specific Heparan Sulfate Proteoglycan Core Protein; Schwartz-Jampel Syndrome 1 (Chondrodystrophic Myotonia); Endorepellin (Domain V Region)
Function
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.
Biological Process
Angiogenesis Source: UniProtKB-KW
Animal organ morphogenesis Source: GO_Central
Brain development Source: ARUK-UCL
Cell differentiation Source: ARUK-UCL
Circulatory system development Source: ARUK-UCL
Inflammatory response Source: ARUK-UCL
Lipid metabolic process Source: ARUK-UCL
Negative regulation of amyloid fibril formation Source: ARUK-UCL
Negative regulation of angiogenesis Source: ARUK-UCL
Negative regulation of cell death Source: ARUK-UCL
Receptor-mediated endocytosis Source: ARUK-UCL
Tissue development Source: GO_Central
Cellular Location
Basement membrane
Involvement in disease
Schwartz-Jampel syndrome (SJS1):
Rare autosomal recessive disorder characterized by permanent myotonia (prolonged failure of muscle relaxation) and skeletal dysplasia, resulting in reduced stature, kyphoscoliosis, bowing of the diaphyses and irregular epiphyses.
Dyssegmental dysplasia Silverman-Handmaker type (DDSH):
The dyssegmental dysplasias are rare, autosomal recessive skeletal dysplasias with anisospondyly and micromelia. There are two recognized types: the severe, lethal DDSH and the milder Rolland-Desbuquois form. Individuals with DDSH also have a flat face, micrognathia, cleft palate and reduced joint mobility, and frequently have an encephalocoele. The endochondral growth plate is short, the calcospherites (which are spherical calcium-phosphorus crystals produced by hypertrophic chondrocytes) are unfused, and there is mucoid degeneration of the resting cartilage.
PTM
Proteolytic processing produces the C-terminal angiogenic peptide, endorepellin. This peptide can be further processed to produce the LG3 peptide.
N- and O-glycosylated. O-glycosylated with core 1 or possibly core 8 glycans. Perlecan contains three heparan sulfate chains. The LG3 peptide contains at least three and up to five potential O-glycosylation sites but no N-glycosylation.
More Infomation

Zhang, W., Lin, Z., Shi, F., Wang, Q., Kong, Y., Ren, Y., ... & Wang, Q. (2022). Hspg2 mutation association with immune checkpoint inhibitor outcome in melanoma and non-small cell lung cancer. Cancers, 14(14), 3495.

De Paolis, V., Maiullari, F., Chirivì, M., Milan, M., Cordiglieri, C., Pagano, F., ... & Parisi, C. (2022). Unusual Association of NF-κB Components in Tumor-Associated Macrophages (TAMs) Promotes HSPG2-Mediated Immune-Escaping Mechanism in Breast Cancer. International Journal of Molecular Sciences, 23(14), 7902.

Lin, P. Y., Hung, J. H., Hsu, C. K., Chang, Y. T., & Sun, Y. T. (2021). A Novel Pathogenic HSPG2 Mutation in Schwartz–Jampel Syndrome. Frontiers in Neurology, 12, 632336.

Zhou, X., Liang, S., Zhan, Q., Yang, L., Chi, J., & Wang, L. (2020). HSPG2 overexpression independently predicts poor survival in patients with acute myeloid leukemia. Cell Death & Disease, 11(6), 492.

Ma, Y., Jin, J., Tong, X., Yang, W., Ren, P., Dai, Y., ... & Zhang, S. (2020). ADAMTS1 and HSPG2 mRNA levels in cumulus cells are related to human oocyte quality and controlled ovarian hyperstimulation outcomes. Journal of assisted reproduction and genetics, 37, 657-667.

Kalscheuer, S., Khanna, V., Kim, H., Li, S., Sachdev, D., DeCarlo, A., ... & Panyam, J. (2019). Discovery of HSPG2 (Perlecan) as a therapeutic target in triple negative breast cancer. Scientific reports, 9(1), 12492.

Apostolidis, S. A., Stifano, G., Tabib, T., Rice, L. M., Morse, C. M., Kahaleh, B., & Lafyatis, R. (2018). Single cell RNA sequencing identifies HSPG2 and APLNR as markers of endothelial cell injury in systemic sclerosis skin. Frontiers in immunology, 9, 2191.

Martinez, J. R., Dhawan, A., & Farach-Carson, M. C. (2018). Modular proteoglycan perlecan/HSPG2: mutations, phenotypes, and functions. Genes, 9(11), 556.

Zai, C. C., Lee, F. H., Tiwari, A. K., Lu, J. Y., Luca, V. D., Maes, M. S., ... & Kennedy, J. L. (2018). Investigation of the HSPG2 gene in tardive dyskinesia–new data and meta-analysis. Frontiers in Pharmacology, 9, 974.

Kazanskaya, G. M., Tsidulko, A. Y., Volkov, A. M., Kiselev, R. S., Suhovskih, A. V., Kobozev, V. V., ... & Grigorieva, E. V. (2018). Heparan sulfate accumulation and perlecan/HSPG2 up-regulation in tumour tissue predict low relapse-free survival for patients with glioblastoma. Histochemistry and cell biology, 149, 235-244.

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For research use only. Not intended for any clinical use.

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