货号：MABT12

品牌：Merck Millipore

规格：100Ug

目录价：￥5513.00

市场价格：￥4686.05

会员价格：￥4410.40

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-Heparan Sulfate Proteoglycan (Perlecan) Antibody, clone 5D7-2E4 | MABT12 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Molecular Weight: 469 kDa calculated View All» Immunogen: Full length recombinant protein corresponding to human Perlecan. View All» Clone: 5D7-2E4 View All» Isotype: IgG1κ View All» Background Information: Perlecan is one of the extracellular matrix forms of heparan sulfate proteoglycans (HSPGs). It is a large multidomain and binds to and cross-links many extracellular matrix (ECM) components and cell-surface molecules. Perlecan is synthesized by vascular endothelial and smooth muscle cells and deposited in the extracellular matrix within all mammalian tissues. HSPGs differ in their protein cores that are thought to determine the location of HSPG in either the cell membrane (syndecan and glypican) or the extracellular matrix (perlecan and dystroglycan). HSPGs found in the brain are dystroglycan, N-syndecan glypican, and perlecan. The composition of HS isolated from brain differs significantly from that of other organs and is developmentally regulated. Thus, differences in protein cores, disaccharide composition, the extent and position of sulfation, and the number of GAG chains diversify chemical structure and functions of HSPGs. The postulated functions of HSPG include cell proliferation, differentiation, adhesion, migration, and morphogenesis. View All» Species Reactivity: Human View All» Species Reactivity Note: Demonstrated to react with human. View All» Application Notes: Immunocytochemistry Analysis: A previous lot was used by an independent laboratory in IC. (courtesy of Whitelock, J. Graduate School of Biomedical Engineering, University of New South Wales, Sydney, Australia)Western Blot Analysis: A previous lot was used by an independent laboratory on a 3-8% Tris-acetate gel in which the band appears above the 460 kDa marker. (courtesy of Whitelock, J. Graduate School of Biomedical Engineering, University of New South Wales, Sydney, Australia) View All» Control: Fresh-frozen normal human tonsil tissue View All» Quality Assurance: Evaluated by Immunohistochemistry in fresh-frozen normal human tonsil tissue.Immunohistochemistry Analysis: 1:300 dilution of the antibody detected Perlecan in fresh-frozen normal human tonsil tissue. View All» Purification Method: Protein G View All» Presentation: Purified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide. View All» Storage Conditions: Stable for 1 year at 2-8°C from date of receipt. View All» UniProt Number: P98160 View All» Entrez Gene Number: NP_005520 View All» Gene Symbol:

• HSPG2

• SJA

• perlecan

• SJS

• HSPG

• PRCAN

• Perlecan

• SJS1

• PLC
  View All» Alternate Names:
  • heparan sulfate proteoglycan 2 (domain V region)

  • perlecan proteoglycan

  • basement membrane-specific heparan sulfate proteoglycan core protein

  • HSPG

  • PLC
    View All» Usage Statement: Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals. View All» Key Applications: Immunohistochemistry View All» Entrez Gene Summary: 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 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. View All» UniProt Summary: FUNCTION: This protein is an integral component of basement membranes. It is responsible for the fixed negative electrostatic charge and is involved in the charge-selective ultrafiltration properties. It serves as an attachment substrate for cells.SIZE: 4391 amino acids; 468825 Da SUBUNIT: Purified perlecan has a strong tendency to aggregate in dimers or stellate structures. It interacts with other basement membrane components such as laminin, prolargin and collagen type IV. Interacts with COL13A1, FGFBP1 and VWA1.SUBCELLULAR LOCATION: Secreted, extracellular space.TISSUE SPECIFICITY: Found in the basement membranes.PTM: N- and O-glycosylated; contains three heparan sulfate chains.DISEASE: Defects in HSPG2 are the cause of Schwartz-Jampel syndrome (SJS1) [MIM:255800]; 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) [MIM:224410]. 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.SIMILARITY: Contains 4 EGF-like domains. & Contains 22 Ig-like C2-type (immunoglobulin-like) domains. & Contains 11 laminin EGF-like domains. & Contains 3 laminin G-like domains. & Contains 3 laminin IV type A domains. & Contains 4 LDL-receptor class A domains. & Contains 1 SEA domain. View All» Product Name: Anti-Heparan Sulfate Proteoglycan (Perlecan), clone 5D7-2E4 View All» Concentration: 1.0 mg/mL View All» Antibody Type: Monoclonal Antibody View All» Qty/Pk: 100 μg View All» Format: Purified View All» Host: Mouse View All»
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