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Basement membrane-specific heparan sulfate proteoglycan core protein

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Below are the list of possible Basement membrane-specific heparan sulfate proteoglycan core protein products. If you cannot find the target and/or product is not available in our catalog, please click here to contact us and request the product or submit your request for custom elisa kit production, custom recombinant protein production or custom antibody production. Custom ELISA Kits, Recombinant Proteins and Antibodies can be designed, manufactured and produced according to the researcher's specifications.
 

Basement membrane-specific heparan sulfate proteoglycan core protein

 Basement membrane-specific heparan sulfate proteoglycan core protein ELISA Kit
 Basement membrane-specific heparan sulfate proteoglycan core protein Recombinant
 Basement membrane-specific heparan sulfate proteoglycan core protein Antibody
Also known as Basement membrane-specific heparan sulfate proteoglycan core protein (HSPG).
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 rol
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es 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; Motility/polarity/chemotaxis; Secreted; Secreted, signal peptide

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

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

Biological Process: brain development; cardiac muscle development; chondrocyte differentiation; embryonic skeletal morphogenesis; endochondral ossification; extracellular matrix organization and biogenesis; protein localization
 Hspg2 ELISA Kit
 Hspg2 Recombinant
 Hspg2 Antibody
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