MAB5428-25UG | Anti-Retinal Pigment Epithelium 65

MAB5428-25UG
25 μg  
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      Description
      Catalogue NumberMAB5428-25UG
      Brand Family Chemicon®
      Trade Name
      • Chemicon
      DescriptionAnti-Retinal Pigment Epithelium 65
      Alternate Names
      • RPE65
      References
      Product Information
      FormatPurified
      Control
      • Eye
      PresentationPurified immunoglobulin. Liquid in 0.02M phosphate buffer, pH 7.6, 0.25M NaCl, and 0.1% sodium azide.
      Applications
      ApplicationAnti-Retinal Pigment Epithelium 65 Antibody detects level of Retinal Pigment Epithelium 65 & has been published & validated for use in ELISA, IH, IP & WB.
      Key Applications
      • ELISA
      • Immunohistochemistry
      • Immunoprecipitation
      • Western Blotting
      Application NotesWestern blot: 1:5,000-1:20,000 on bovine RPE membranes using ECL. Suggested dilution buffer is TBS containing 10% calf serum, 0.25% T-20, 1M D-glucose with 10% glycerol. Suggested blocking buffer is TBS containing 2% BSA and 0.5% Tween 20. Preferred gel percentage is 10%.

      Immunohistochemistry on frozen tissue sections: 1:250-1:500

      Immunoprecipitation: 20 μg of antibody in a reaction volume of 500 μL.

      Immunoaffinity purification

      ELISA

      Optimal working dilutions must be determined by end user.
      Biological Information
      ImmunogenBovine RPE microsomal membranes.
      Clone401.8B11.3D9
      ConcentrationPlease refer to lot specific datasheet.
      HostMouse
      SpecificityReacts with Retinal Pigment Epithelium 65 (RPE65). On bovine RPE membranes the antibody recognizes a protein with a molecular weight of ~65 kDa.
      IsotypeIgG
      Species Reactivity
      • Bovine
      • Human
      • Mouse
      • Xenopus
      Antibody TypeMonoclonal Antibody
      Entrez Gene Number
      Entrez Gene SummaryThis gene encodes a protein which is located in the retinal pigment epithelium and is involved in the production of 11-cis retinal and in visual pigment regeneration. There are two forms of this protein, a soluble form called sRPE65, and a palmitoylated, membrane-bound form known as mRPE65. mRPE65 serves as the palmitoyl donor for lecithin retinol acyl transferase (LRAT), the enzyme that catalyzes the vitamin A to all trans retinol step of the chromophore regeneration process. Both mRPE65 and sRPE65 also serve as regulatory proteins, with the ratio and concentrations of these molecules playing a role in the inhibition of 11-cis retinal synthesis. Mutations in this gene have been associated with Leber congenital amaurosis type 2 (LCA2) and retinitis pigmentosa.
      Gene Symbol
      • RPE65
      • mRPE65
      • rd12
      • LCA2
      • p63
      • RP20
      • sRPE65
      Purification MethodProtein A Purfied
      UniProt Number
      UniProt SummaryFUNCTION: SwissProt: Q16518 # Plays important roles in the production of 11-cis retinal and in visual pigment regeneration. The soluble form binds vitamin A (all-trans-retinol), making it available for LRAT processing to all-trans-retinyl ester. The membrane form, palmitoylated by LRAT, binds all-trans-retinyl esters, making them available for IMH (isomerohydrolase) processing to all-cis- retinol. The soluble form is regenerated by transferring its palmitoyl groups onto 11-cis-retinol, a reaction catalyzed by LRAT (By similarity).
      SIZE: 533 amino acids; 60948 Da
      SUBCELLULAR LOCATION: Cytoplasm (By similarity). Cell membrane; Lipid-anchor (By similarity). Note=Attached to the membrane by a lipid anchor when palmitoylated (membrane form), soluble when unpalmitoylated (By similarity).
      TISSUE SPECIFICITY: Retinal pigment epithelium specific.
      PTM: Palmitoylation by LRAT regulates ligand binding specificity; the palmitoylated form (membrane form) specifically binds all- trans-retinyl-palmitate, while the soluble unpalmitoylated form binds all-trans-retinol (vitamin A) (By similarity).
      DISEASE: SwissProt: Q16518 # Defects in RPE65 are the cause of Leber congenital amaurosis type 2 (LCA2) [MIM:204100]. LCA designates a clinically and genetically heterogeneous group of childhood retinal degenerations, generally inherited in an autosomal recessive manner. Affected infants have little or no retinal photoreceptor function as tested by electroretinography. LCA represents the most common genetic cause of congenital visual impairment in infants and children. & Defects in RPE65 are the cause of retinitis pigmentosa type 20 (RP20) [MIM:180069]. RP leads to degeneration of retinal photoreceptor cells. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well. RP20 inheritance is autosomal dominant.
      SIMILARITY: SwissProt: Q16518 ## Belongs to the carotenoid oxygenase family.
      Molecular Weight~ 65 kDa
      Physicochemical Information
      Dimensions
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality AssuranceTested
      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 ConditionsMaintain for 1 year at 2–8°C from date of shipment. Aliquot to avoid repeated freezing and thawing. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
      Packaging Information
      Material Size25 μg
      Transport Information
      Supplemental Information
      Specifications

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