catalog number :
MBS732716
products type :
ELISA Kit
products full name :
Guinea pig Heparan Sulphate Proteoglycan ELISA Kit
products short name :
Heparan Sulphate Proteoglycan
other names :
heparan sulfate proteoglycan core protein, partial; Basement membrane-specific heparan sulfate proteoglycan core protein; basement membrane-specific heparan sulfate proteoglycan core protein; perlecan proteoglycan; endorepellin (domain V region); heparan sulfate proteoglycan 2; Perlecan; PLC
products gene name :
HSPG
other gene names :
HSPG2; HSPG2; PLC; SJA; SJS; HSPG; SJS1; PRCAN; HSPG; PLC
uniprot entry name :
PGBM_HUMAN
specificity :
This assay has high sensitivity and excellent specificity for detection of HSPG. No significant cross-reactivity or interference between HSPG and analogues was observed. NOTE: Limited by current skills and knowledge, it is impossible for us to complete the cross-reactivity detection between HSPG and all the analogues, therefore, cross reaction may still exist in some cases.
storage stability :
Store all reagents at 2-8 degree C.
other info1 :
Samples: Serum, plasma, Cell Culture Supernatants, body fluid and tissue homogenate. Assay Type: Competitive. Sensitivity: 1.0 pg/mL.
other info2 :
Intended Uses: This HSPG ELISA kit is a 1.5 hour solid-phase ELISA designed for the quantitative determination of Guinea pig HSPG. This ELISA kit for research use only, not for therapeutic or diagnostic applications!
products categories :
Cardiovascular
products description :
Principle of the assay: HSPG ELISA kit applies the competitive enzyme immunoassay technique utilizing a monoclonal anti-HSPG antibody and an HSPG-HRP conjugate. The assay sample and buffer are incubated together with HSPG-HRP conjugate in pre-coated plate for one hour. After the incubation period, the wells are decanted and washed five times. The wells are then incubated with a substrate for HRP enzyme. The product of the enzyme-substrate reaction forms a blue colored complex. Finally, a stop solution is added to stop the reaction, which will then turn the solution yellow. The intensity of color is measured spectrophotometrically at 450nm in a microplate reader. The intensity of the color is inversely proportional to the HSPG concentration since HSPG from samples and HSPG-HRP conjugate compete for the anti-HSPG antibody binding site. Since the number of sites is limited, as more sites are occupied by HSPG from the sample, fewer sites are left to bind HSPG-HRP conjugate. A standard curve is plotted relating the intensity of the color (O.D.) to the concentration of standards. The HSPG concentration in each sample is interpolated from this standard curve.
ncbi acc num :
AAB21121.2
ncbi mol weight :
468,830 Da
ncbi pathways :
A Tetrasaccharide Linker Sequence Is Required For GAG Synthesis Pathway (645305); Amyloids Pathway (366238); Chondroitin Sulfate/dermatan Sulfate Metabolism Pathway (645308); Chylomicron-mediated Lipid Transport Pathway (106157); Degradation Of The Extracellular Matrix Pathway (576263); Disease Pathway (530764); Diseases Associated With Visual Transduction Pathway (771581); ECM-receptor Interaction Pathway (83068); ECM-receptor Interaction Pathway (479); Extracellular Matrix Organization Pathway (576262)
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 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]
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: Motility/polarity/chemotaxis; Secreted; Cell adhesion; Secreted, signal peptide. 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; pathogenesis; lipoprotein metabolic process; embryonic skeletal morphogenesis; chondroitin sulfate metabolic process; extracellular matrix disassembly; glycosaminoglycan biosynthetic process; glycosaminoglycan catabolic process; protein localization; carbohydrate metabolic process; chondrocyte differentiation; angiogenesis; brain development; retinoid metabolic process; endochondral ossification. Disease: Schwartz-jampel Syndrome, Type 1; Dyssegmental Dysplasia, Silverman-handmaker Type