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HSPG2

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. [provided by RefSeq, May 2014]
Full Name
Heparan Sulfate Proteoglycan 2
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.

Anti-HSPG2 antibodies

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Target: HSPG2
Host: Mouse
Antibody Isotype: IgG
Specificity: Human
Clone: CBFYH-2474
Application*: E
Target: HSPG2
Host: Mouse
Antibody Isotype: IgG
Specificity: Human
Clone: CBFYH-2473
Application*: WB, IC, C, E
Target: HSPG2
Host: Mouse
Antibody Isotype: IgG1
Specificity: Cattle
Clone: CBFYH-2472
Application*: E, C, IP, WB
Target: HSPG2
Host: Mouse
Antibody Isotype: IgG1
Specificity: Cattle, Human
Clone: CBFYH-2471
Application*: WB, IP, E, P, C, AP, IF
Target: HSPG2
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Cattle
Clone: CBFYH-2470
Application*: E, C, P, IP, WB
Target: HSPG2
Host: Mouse
Antibody Isotype: IgG1
Specificity: Cattle, Human
Clone: CBFYH-2469
Application*: E, IH, IP, WB
Target: HSPG2
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBFYH-2468
Application*: E, C, P, WB
Target: HSPG2
Host: Mouse
Antibody Isotype: IgG
Specificity: Human
Clone: CBFYH-2466
Application*: E, WB
Target: HSPG2
Host: Rat
Antibody Isotype: IgG2a, κ
Specificity: Cattle, Human, Mouse
Clone: CBFYH-2465
Application*: IC
Target: HSPG2
Host: Rat
Antibody Isotype: IgG2a, κ
Specificity: Human
Clone: CBFYH-2464
Application*: IC, IH, IP, WB
Target: HSPG2
Host: Rat
Antibody Isotype: IgG2a
Specificity: Cattle, Human, Mouse, Rat
Clone: CBFYH-2459
Application*: WB, IP, IH, IC
Target: HSPG2
Host: Mouse
Antibody Isotype: IgG1
Specificity: Sheep, Cattle, Human
Clone: CBFYH-2458
Application*: WB, IP, E, P, C, AP
Target: HSPG2
Host: Rat
Antibody Isotype: IgG2a
Specificity: Mouse, Cattle, Rat, Human
Clone: CBFYH-2457
Application*: IC, C, P, IP, WB
Target: HSPG2
Host: Rat
Antibody Isotype: IgG2a, κ
Specificity: Mouse
Clone: CBFYH-2456
Application*: IF, C
Target: HSPG2
Host: Mouse
Antibody Isotype: IgG1
Specificity: Cattle
Clone: CBFYH-2455
Application*: P, WB
Target: HSPG2
Host: Rat
Antibody Isotype: IgG2a
Specificity: Mouse, Rat, Cattle, Human, Pig, Fish
Clone: A7L6
Application*: WB, IP, P, C, IC
Target: HSPG2
Host: Rat
Antibody Isotype: IgG2a, κ
Specificity: Mouse
Clone: CBFYH-2460
Application*: IF, IH
Target: HSPG2
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBFYH-2463
Application*: E, IH, WB
Target: HSPG2
Host: Rat
Antibody Isotype: IgG2a, κ
Specificity: Mouse
Clone: CBFYH-2461
Application*: IC, IH, IP, WB
Target: HSPG2
Host: Mouse
Antibody Isotype: IgG
Specificity: Human
Clone: CBFYH-2467
Application*: E, IC, WB
Target: HSPG2
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBFYE-1468
Application*: WB
Target: HSPG2
Host: Rat
Antibody Isotype: IgG2a, κ
Specificity: Human, Mouse, Pig, Fish, Monkey
Clone: CBFYE-0130
Application*: F, P, IF
Target: HSPG2
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Human, Cattle
Clone: CBFYE-0129
Application*: E, IH, IP, C
Target: HSPG2
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Chicken
Clone: 5C9
Application*: E
Target: HSPG2
Host: Rat
Antibody Isotype: IgG2a, κ
Specificity: Cattle, Human, Monkey, Mouse, Fish
Clone: A341
Application*: FC, IF, IHC
Target: HSPG2
Host: Rat
Antibody Isotype: IgG2a, κ
Specificity: Cattle, Human, Monkey, Mouse, Fish
Clone: A340
Application*: FC, IF, IHC
Target: HSPG2
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human, Cattle
Clone: A76
Application*: E, WB, C, P, IP, AP
Target: HSPG2
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human, Cattle
Clone: A71
Application*: E, IH, IP
Target: HSPG2
Host: Rat
Antibody Isotype: IgG2a
Specificity: Rat, Human, Mouse, Cattle
Clone: A7L6
Application*: IC, IH, C, P, WB
For Research Use Only. Not For Clinical Use.
(P): Predicted
* Abbreviations
IFImmunofluorescence
IHImmunohistochemistry
IPImmunoprecipitation
WBWestern Blot
EELISA
MMicroarray
CIChromatin Immunoprecipitation
FFlow Cytometry
FNFunction Assay
IDImmunodiffusion
RRadioimmunoassay
TCTissue Culture
GSGel Supershift
NNeutralization
BBlocking
AActivation
IInhibition
DDepletion
ESELISpot
DBDot Blot
MCMass Cytometry/CyTOF
CTCytotoxicity
SStimulation
AGAgonist
APApoptosis
IMImmunomicroscopy
BABioassay
CSCostimulation
EMElectron Microscopy
IEImmunoelectrophoresis
PAPeptide Array
ICImmunocytochemistry
PEPeptide ELISA
MDMeDIP
SHIn situ hybridization
IAEnzyme Immunoassay
SEsandwich ELISA
PLProximity Ligation Assay
ECELISA(Cap)
EDELISA(Det)
BIBioimaging
IOImmunoassay
LFLateral Flow Immunoassay
LALuminex Assay
CImmunohistochemistry-Frozen Sections
PImmunohistologyp-Paraffin Sections
ISIntracellular Staining for Flow Cytometry
MSElectrophoretic Mobility Shift Assay
RIRNA Binding Protein Immunoprecipitation (RIP)
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