catalog number :
MBS601511
products full name :
Heparan Sulfate Proteoglycan, Large
products short name :
Heparan Sulfate Proteoglycan, Large
products name syn :
Anti -Heparan Sulfate Proteoglycan, Large
other names :
heparan sulfate proteoglycan; Basement membrane-specific heparan sulfate proteoglycan core protein; basement membrane-specific heparan sulfate proteoglycan core protein; OTTHUMP00000002765; perlecan proteoglycan; endorepellin (domain V region); heparan sulfate proteoglycan 2; Perlecan
other gene names :
HSPG2; HSPG2; PLC; SJA; SJS; HSPG; SJS1; PRCAN
uniprot entry name :
PGBM_HUMAN
reactivity :
Bovine, Human, Mouse, Rat
specificity :
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
purity :
Purified. Purified
form :
Supplied as a liquid in PBS, 0.09% sodium azide.
storage stability :
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.
tested application :
Western Blot (WB), Immunoprecipitation (IP), Immunohistochemistry (IHC), Immunocytochemistry (ICC)
app notes :
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.
other info1 :
Immunogen: Derived from the Engelbreth-Holm-Swarm (EHS) tumor matrix containing laminin, entactin and Heparan Sulfate Proteoglycan.
other info2 :
Hybridoma: X63Ag8.653 mouse myeloma cells with spleen cells from a Fisher rat.
products categories :
Antibodies; Abs to Carbohydrates, Glycoproteins
products description :
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.
ncbi mol weight :
468,830 Da
ncbi pathways :
Amyloids Pathway (366238); Chylomicron-mediated Lipid Transport Pathway (106157); ECM-receptor Interaction Pathway (83068); ECM-receptor Interaction Pathway (479); Lipid Digestion, Mobilization, And Transport Pathway (106111); Lipoprotein Metabolism Pathway (106156); Metabolism Of Lipids And Lipoproteins Pathway (160976)
ncbi summary :
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.
uniprot summary :
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