Anti -Heparan-Sulfate Proteoglycan

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

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

Monoclonal

IgG1,k

2H7/G11

Mouse

Recognizes human Heparan-Sulfate-Proteoglycan. specific for a core protein epitope of a human small basement membrane heparan sulfate proteo-glycan (HS-PG).

Affinity Purified
Purified by Protein G affinity chromatography.

Supplied as a lyophilized powder in 50mM TBS, pH 7.2.

Lyophilized powder may be stored at 4 degree C for short-term only. Reconstitute to nominal volume by adding sterile 40-50% glycerol and store at -20 degree C. Reconstituted product is stable for 12 months at -20 degree C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

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.

The principal molecular structure of basement membranes has been elucidated during the past two decades and it was shown that for example in the glomerular basement membrane (GBM) HS-PG are responsible for the selective filtration process. Removal of HS-PG will lead to proteinuria. Whereas previously perlecan was the only known basement membrane HS-PG, there is now evidence that (at least two) other basement membrane HS-PG do exist: Agrin, originally discovered as an important component of the neuromuscular junction and a novel small HS-PG, that was isolated from human aorta and kidney. This HS-PG, with a molecular weight of 80-200kD (aorta) and 30-160kD (kidney) and a core protein size of 24kD or 22kD, respectively, was localized by immunohisto-hemistry to the basement membrane. Amino acid sequence analysis of tryptic peptides indicate, that this small HS-PG is clearly distinct from perlecan and agrin.

Antibodies; Abs to Carbohydrates, Glycoproteins

ELISA (EL/EIA), Western Blot (WB), Immunohistochemistry (IHC)

Suitable for use in ELISA, Western Blotting, Immunohistochemistry.
Dilution: ELISA: 1ug/ml
Western Blotting: 1ug/ml
Immunohistochemistry: 1ug/ml

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

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

Cellular Component: extracellular matrix; lysosomal lumen; extracellular space; focal adhesion; Golgi lumen; extracellular region; plasma membrane; 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; glycosaminoglycan biosynthetic process; extracellular matrix disassembly; protein localization; glycosaminoglycan catabolic process; carbohydrate metabolic process; chondrocyte differentiation; angiogenesis; brain development; retinoid metabolic process; endochondral ossification

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

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