Anti -Heparan Sulfate Proteoglycan, Large

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

Chromosome: 1; NC_000001.10 (22148737..22222804, complement). Location: 1p36.1-p34

Monoclonal

IgG2a

4A49

Rat

Recognizes the large Heparan Sulfate Proteoglycan. Reacts with domain IV of the core protein of the large heparan sulfate proteoglycan or perlecan. Reactivity is independent of the galactosaminoglycan moieties. The epitope is therefore not sensitive to heparitinase treatment. Species Crossreactivity: human, mouse, rat and bovine

Purified
Purified

Supplied as a liquid in PBS, 0.09% sodium azide.

1mg/ml (lot specific)

May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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.

Proteoglycans are macromolecules consisting of a variety of core proteins with covalently attached one or several polysaccharide chains of the glycosaminoglycan type (heparan sulfate, heparin, chondroitin sulfate, dermatan sulfate or keratan sulfate). Two forms of basement membrane heparan sulfate proteoglycan (HSPG) have been identified. One with a large core protein (> 400kD) and one with a small core protein (30kD). The large HSPG is probably the most abundant basement membrane proteoglycan. It is located predominantly in the lamina lucida, where it forms clustered aggregates and interacts with other basement membrane components to form the matrix. In addition, it also plays a critical role in attachment of cells to the basal membrane via integrin receptors.

Antibodies; Abs to Carbohydrates, Glycoproteins

Western Blot (WB), Immunoprecipitation (IP), Immunohistochemistry (IHC), Immunocytochemistry (ICC)

Suitable for use in Western Blot, Immunoprecipitation, Immunohistochemistry and Immunocytochemistry.
Dilution: Western Blot: 1:100-1:1000
Immunohistochemistry (Frozen, paraffin): 1:25-1:200 with avidin-biotinylated horseradish peroxidase complex (ABC) as a detecting reagent.
Optimum dilutions to be determined by researcher.

Immunohistochemistry on frozen section of Human kidney showing strong reactivity in the extracellular matrix and basement membrane using MBS601511

468,830 Da[Similar Products]

heparan sulfate proteoglycan 2

basement membrane-specific heparan sulfate proteoglycan core protein; OTTHUMP00000002765; perlecan proteoglycan; endorepellin (domain V region)

Basement membrane-specific heparan sulfate proteoglycan core protein

PGBM_HUMAN

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 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.

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

Chromosomal Location of Human Ortholog: 1p36.1-p34

Cellular Component: extracellular matrix; extracellular space; lysosomal lumen; focal adhesion; Golgi lumen; plasma membrane; extracellular region; basal lamina

Molecular Function: protein C-terminus binding; protein binding; metal ion binding

Biological Process: cardiac muscle development; phototransduction, visible light; extracellular matrix organization and biogenesis; glycosaminoglycan metabolic process; lipoprotein metabolic process; pathogenesis; embryonic skeletal morphogenesis; chondroitin sulfate metabolic process; extracellular matrix disassembly; glycosaminoglycan biosynthetic process; protein localization; glycosaminoglycan catabolic process; carbohydrate metabolic process; chondrocyte differentiation; brain development; angiogenesis; retinoid metabolic process; endochondral ossification

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

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.