ELISA/assay

Human Heparan Sulphate Proteoglycan ELISA Kit

MBS733802

48-Strip-Wells-(Comp

470 USD

ELISA Kit

Human Heparan Sulphate Proteoglycan ELISA Kit

Heparan Sulphate Proteoglycan

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

HSPG

HSPG2; HSPG2; PLC; SJA; SJS; HSPG; SJS1; PRCAN; HSPG; PLC

PGBM_HUMAN

Human

Store all reagents at 2-8 degree C.

Assay Type: Competitive or Sandwich. Samples: Serum, plasma, Cell Culture Supernatants, body fluid and tissue homogenate. Sensitivity: 0.1 ng/mL.

Cardiovascular

Intended Uses: This HSPG ELISA kit is a 1.5 hour solid-phase ELISA designed for the quantitative determination of Human HSPG. This ELISA kit for research use only, not for therapeutic or diagnostic applications!. Principle of the Assay: HSPG ELISA kit applies the quantitative sandwich enzyme immunoassay technique. The microtiter plate has been pre-coated with a monoclonal antibody specific for HSPG. Standards or samples are then added to the microtiter plate wells and HSPG if present, will bind to the antibody pre-coated wells. In order to quantitatively determine the amount of HSPG present in the sample, a standardized preparation of horseradish peroxidase (HRP)-conjugated polyclonal antibody, specific for HSPG are added to each well to "sandwich" the HSPG immobilized on the plate. The microtiter plate undergoes incubation, and then the wells are thoroughly washed to remove all unbound components. Next, substrate solutions are added to each well. The enzyme (HRP) and substrate are allowed to react over a short incubation period. Only those wells that contain HSPG and enzyme-conjugated antibody will exhibit a change in color. The enzyme-substrate reaction is terminated by addition of a sulphuric acid solution and the color change is measured spectrophotometrically at a wavelength of 450 nm. 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.

13242858

AAB21121.2

468,830 Da

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)

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

48-Strip-Wells-(Competitive)

470 USD

48-Strip-Wells-(Sandwich)

470

96-Strip-Wells-(Competitive)

675

96-Strip-Wells-(Sandwich)

675