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MAB1948P | Anti-Heparan Sulfate Proteoglycan (Perlecan) Antibody, clone A7L6

MAB1948P
100 µg  
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      Replacement Information

      Ofertas especiales

      Tabla espec. clave

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      H IHC, ICC, WB, IP R Purified Monoclonal Antibody
      Description
      Catalogue NumberMAB1948P
      ReplacesMAB1948
      DescriptionAnti-Heparan Sulfate Proteoglycan (Perlecan) Antibody, clone A7L6
      Alternate Names
      • heparan sulfate proteoglycan 2
      • Schwartz-Jampel syndrome 1 (chondrodystrophic myotonia)
      • endorepellin (domain V region)
      • perlecan proteoglycan
      • heparan sulfate proteoglycan of basement membrane
      Background InformationHeparan sulfate proteoglycans (HSPGs) are found on cell surfaces and in the extracelluar matrix of all mammalian tissues, particularly in the core of the basement membrane. HSPGs found in the brain are dystroglycan, N-syndecan, glypican, and perlecan. The composition of HSPGs differs in the protein core, and is thought to determine the location of HSPG in the cell membrane (syndecan and glypican) and extracellular matrix (perlecan and dystroglycan). The postulated functions of HSPG include cell proliferation, differentiation, adhesion, migration, and morphogenesis.
      Heparan Sulfate Proteoglycan antibodies, in tissues, react strongly and uniformly with basement membranes. Clone A7L6 recognizes domain IV of the core protein of the large heparan sulphate proteoglycan or perlecan. The reactivity is independent of the galactosaminoglycan moieties; therefore, the epitope is not sensitive to heparinase treatment.
      References
      Product Information
      FormatPurified
      Control
      • HeLa and A431 cells
      PresentationPurified rat monoclonal IgG2aκ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
      Applications
      ApplicationDetect Heparan Sulfate Proteoglycan (Perlecan) using this Anti-Heparan Sulfate Proteoglycan (Perlecan) Antibody, clone A7L6 validated for use in IH, IC, WB & IP.
      Key Applications
      • Immunohistochemistry
      • Immunocytochemistry
      • Western Blotting
      • Immunoprecipitation
      Application NotesImmunohistochemistry Analysis: 1:100 dilution from a previous lot detected Heparan Sulfate Proteoglycan in large cell carcinoma tissue.

      Western Blot Analysis: A previous lot was used in independent laboratories in WB. (Hagen, 1993; Brown, 1999).

      Immunoprecipitation Analysis: A previous lot was used by an independent laboratory in IP. (Couchman, 1989).
      Biological Information
      ImmunogenHeparan Sulfate Proteoglycan from EHS mouse tumor
      EpitopeHSPG core protein
      CloneA7L6
      ConcentrationPlease refer to the Certificate of Analysis for the lot-specific concentration.
      HostRat
      SpecificityThe antibody recognizes a high molecular weight core protein of Heparan Sulfate Proteoglycan (Perlecan). No cross reactivity to laminin, collagen IV, entactin/nidogen, or fibronectin (Horiguchi, 1989).
      IsotypeIgG2aκ
      Species Reactivity
      • Human
      Antibody TypeMonoclonal Antibody
      Entrez Gene Number
      Entrez Gene SummaryThis 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.
      Gene Symbol
      • HSPG2
      • SJS
      • PRCAN
      • SJS1
      • SJA
      • perlecan
      • HSPG
      • Perlecan
      • PLC
      Purification MethodProtein G Purified
      UniProt Number
      UniProt SummaryFUNCTION: 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.
      Physicochemical Information
      Dimensions
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality AssuranceEvaluated by Immunocytochemistry in HeLa and A431 cells.

      Immunocytochemistry Analysis: 1:500 dilution of this antibody detected Heparan Sulfate Proteoglycan in HeLa and A431 cells.
      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.
      Storage and Shipping Information
      Storage ConditionsStable for 1 year at 2-8°C from date of receipt.
      Packaging Information
      Material Size100 µg
      Transport Information
      Supplemental Information
      Specifications

      Documentation

      Ficha datos de seguridad (MSDS)

      Título

      Ficha técnica de seguridad del material (MSDS) 

      Certificados de análisis

      CargoNúmero de lote
      Anti-Heparan Sulfate Proteoglycan (Perlecan), clone A7L6 - 2145112 2145112
      Anti-Heparan Sulfate Proteoglycan (Perlecan), clone A7L6 - 2455612 2455612
      Anti-Heparan Sulfate Proteoglycan (Perlecan), clone A7L6 - 2049814 2049814
      Anti-Heparan Sulfate Proteoglycan (Perlecan), clone A7L6 - 2070001 2070001
      Anti-Heparan Sulfate Proteoglycan (Perlecan), clone A7L6 - 2216910 2216910
      Anti-Heparan Sulfate Proteoglycan (Perlecan), clone A7L6 - 2302254 2302254
      Anti-Heparan Sulfate Proteoglycan (Perlecan), clone A7L6 - JBC1787362 JBC1787362
      Anti-Heparan Sulfate Proteoglycan (Perlecan), clone A7L6 - NG1743563 NG1743563
      Anti-Heparan Sulfate Proteoglycan (Perlecan), clone A7L6 - NG1820258 NG1820258
      Anti-Heparan Sulfate Proteoglycan (Perlecan), clone A7L6 - NG1845606 NG1845606
      Anti-Heparan Sulfate Proteoglycan (Perlecan), clone A7L6 - NG1873220 NG1873220
      Anti-Heparan Sulfate Proteoglycan (Perlecan), clone A7L6 - NRG1948P NRG1948P
      Anti-Heparan Sulfate Proteoglycan (Perlecan), clone A7L6 -2517844 2517844
      Anti-Heparan Sulfate Proteoglycan (Perlecan), clone A7L6 -2573553 2573553
      Anti-Heparan Sulfate Proteoglycan (Perlecan), clone A7L6 -2632992 2632992
      Anti-Heparan Sulfate Proteoglycan (Perlecan), clone A7L6 -2746422 2746422
      Anti-Heparan Sulfate Proteoglycan -2700974 2700974
      Anti-Heparan Sulfate Proteoglycan -2798596 2798596

      Referencias bibliográficas

      Visión general referenciasPub Med ID
      Reduced sulfation of chondroitin sulfate but not heparan sulfate in kidneys of diabetic db/db mice.
      Reine, TM; Grøndahl, F; Jenssen, TG; Hadler-Olsen, E; Prydz, K; Kolset, SO
      The journal of histochemistry and cytochemistry : official journal of the Histochemistry Society 61 606-16 2013

      Mostrar resumen
      23757342 23757342
      Mouse fukutin deletion impairs dystroglycan processing and recapitulates muscular dystrophy.
      Beedle, Aaron M, et al.
      J. Clin. Invest., 122: 3330-42 (2012) 2012

      Mostrar resumen
      22922256 22922256