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

Store all reagents at 2-8 degree C.

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Small volumes of HSPG elisa kit 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.

MBS269559 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Heparan Sulfate Proteoglycan (HSPG) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing HSPG. The ELISA analytical biochemical technique of the MBS269559 kit is based on HSPG antibody-HSPG antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect HSPG antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, HSPG. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

468,830 Da

heparan sulfate proteoglycan 2

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

Basement membrane-specific heparan sulfate proteoglycan core protein

HSPG; PLC??[Similar Products]

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

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

Cellular Component: extracellular matrix; extracellular space; lysosomal lumen; 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; pathogenesis; lipoprotein metabolic process; embryonic skeletal morphogenesis; chondroitin sulfate metabolic process; glycosaminoglycan biosynthetic process; extracellular matrix disassembly; 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

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