For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

The antibody is a rabbit polyclonal antibody raised against HSPG2. It has been selected for its ability to recognize HSPG2 in immunohistochemical staining andwestern blotting.

Affinity Chromatography

Supplied as solution form in PBS, pH7.4, containing 0.02% NaN3,50% glycerol.

200ug/ml (lot specific)

Store at 4 degree C for frequent use. Stored at -20 degree C to -80 degree C in a manual defrost freezer for one year without detectable loss of activity. Avoid repeated freeze-thaw cycles.

Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.

Manufactured in a lab with traceable raw materials. Bulk orders can typically be prepared to the customera??s specifications, please inquire.

Small volumes of anti-HSPG2 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Immunocytochemistry (ICC), Immunohistochemistry (IHC) - Formalin/Paraffin, ELISA (EIA), Western Blot (WB)

Western blotting: 1:100-400
Immunocytochemistry in formalin fixed cells: 1:100-500
Immunohistochemistry in formalin fixed frozen section: 1:100-500
Immunohistochemistry in paraffin section: 1:50-200
Enzyme-linked Immunosorbent Assay: 1:100-200

Western Blot: Sample: Porcine Heart lysate; Primary Ab: 5ug/ml Rabbit Anti-Human HSPG2 Antibody Second Ab: 0.2ug/mL?HRP-Linked Caprine Anti-Rabbit IgG Polyclonal Antibody

Western Blot: Sample: Recombinant protein.

DAB staining on IHC-P. Samples: Human Tissue)

468,830 Da

heparan sulfate proteoglycan 2

basement membrane-specific heparan sulfate proteoglycan core protein

Basement membrane-specific heparan sulfate proteoglycan core protein

HSPG; PLC??[Similar Products]

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]

HSPG2: 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. Defects in HSPG2 are the cause of Schwartz-Jampel syndrome (SJS1); a 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. Defects in HSPG2 are the cause of 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.

Protein type: Cell adhesion; Motility/polarity/chemotaxis; Secreted; Secreted, signal peptide

Chromosomal Location of Human Ortholog: 1p36.12

Cellular Component: basement membrane; extracellular exosome; extracellular matrix; extracellular region; extracellular space; focal adhesion; Golgi lumen; lysosomal lumen; plasma membrane; plasma membrane protein complex

Molecular Function: calcium ion binding; low-density lipoprotein receptor binding; protein binding; protein C-terminus binding

Biological Process: angiogenesis; brain development; cardiovascular system development; cell differentiation; cell growth; cellular protein metabolic process; extracellular matrix disassembly; extracellular matrix organization; glycosaminoglycan biosynthetic process; glycosaminoglycan catabolic process; glycosaminoglycan metabolic process; inflammatory response; lipid metabolic process; negative regulation of angiogenesis; receptor-mediated endocytosis; retinoid metabolic process

Disease: Dyssegmental Dysplasia, Silverman-handmaker Type; Schwartz-jampel Syndrome, Type 1

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