Western Blot (WB)
((Figure. Western Blot; Sample: Recombinant protein.))
Rabbit anti-Human Heparan Sulfate Proteoglycan (HSPG) Polyclonal Antibody | anti-HSPG antibody
Biotin-Linked Antibody to Heparan Sulfate Proteoglycan (HSPG)
Immunocytochemistry in formalin fixed cells: 1:100-500
Immunohistochemistry in formalin fixed frozen section: 1:100-500
Immunohistochemistry in paraffin section: 1:50-200
Enzyme-linked Immunosorbent Assay: 1:100-200
Western Blot (WB)
((Figure. Western Blot; Sample: Recombinant protein.))
Western Blot (WB)
((Figure. Western Blot; Sample: Recombinant protein.))
Sequence
Sequence
NCBI and Uniprot Product Information
NCBI Description
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 Description
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; Secreted, signal peptide; Motility/polarity/chemotaxis
Chromosomal Location of Human Ortholog: 1p36.1-p34
Cellular Component: extracellular matrix; lysosomal lumen; extracellular space; 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; extracellular matrix disassembly; glycosaminoglycan biosynthetic process; protein localization; glycosaminoglycan catabolic process; carbohydrate metabolic process; chondrocyte differentiation; angiogenesis; brain development; retinoid metabolic process; endochondral ossification
Disease: Schwartz-jampel Syndrome, Type 1; Dyssegmental Dysplasia, Silverman-handmaker Type
Research Articles on HSPG
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Product Notes
The HSPG hspg2 (Catalog #AAA2004505) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The immunogen sequence is Ala347~Asp466. The Biotin-Linked Antibody to Heparan Sulfate Proteoglycan (HSPG) reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's Heparan Sulfate Proteoglycan (HSPG) can be used in a range of immunoassay formats including, but not limited to, Immunohistochemistry (IHC), ELISA (EIA), Western Blot (WB). Western blotting: 1:100-400 Immunocytochemistry in formalin fixed cells: 1:100-500 Immunohistochemistry in formalin fixed frozen section: 1:100-500 Immunohistochemistry in paraffin section: 1:50-200 Enzyme-linked Immunosorbent Assay: 1:100-200. Researchers should empirically determine the suitability of the HSPG hspg2 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "Heparan Sulfate Proteoglycan (HSPG), Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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