Mouse Basement membrane-specific heparan sulfate proteoglycan core protein (HSPG2) ELISA kit; RP11-132G19.2; PLC; PRCAN; SJA; SJS; SJS1; Schwartz-Jampel syndrome 1 (chondrodystrophic myotonia) ; endorepellin (domain V region) ; heparan sulfate proteoglycan of basement membrane; perlecan proteogly; heparan sulfate proteoglycan 2

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 HSPG2 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.

MBS9327167 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the heparan sulfate proteoglycan 2 (HSPG2) 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 HSPG2. The ELISA analytical biochemical technique of the MBS9327167 kit is based on HSPG2 antibody-HSPG2 antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect HSPG2 antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, HSPG2. 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(%).

398,294 Da

perlecan (heparan sulfate proteoglycan 2)

basement membrane-specific heparan sulfate proteoglycan core protein; HSPG

Basement membrane-specific heparan sulfate proteoglycan core protein

HSPG??[Similar Products]

PGBM_MOUSE

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

Cellular Component: extracellular matrix; proteinaceous extracellular matrix; extracellular space; focal adhesion; extracellular region; basement membrane; basal lamina

Molecular Function: protein C-terminus binding; protein binding; protease binding; metal ion binding

Biological Process: cardiac muscle development; extracellular matrix organization and biogenesis; protein localization; chondrocyte differentiation; brain development; angiogenesis; embryonic skeletal morphogenesis; endochondral ossification

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