530918 | PTC-124 - CAS 775304-57-9 - Calbiochem

530918
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      概要

      Replacement Information

      主要スペック表

      Empirical FormulaCAS #
      C₁₅H₉FN₂O₃ 775304-57-9

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      5.30918.0001
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          ガラスビン 25 mg
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          Description
          OverviewA cell-permeable, non-toxic, orally bioavailable oxadiazole compound that is reported to promote read-through of premature, but not normal, translation termination codons and thereby suppress premature termination in protein synthesis caused by nonsense mutations (in ~10 to 100 nM with maximal activity reported at 3 µM). Promotes the suppression of nonsense alleles of the dystrophin gene in primary human muscle cells and in a mouse model of Duchenne muscular dystrophy (mdx). Also reported to rescue striated muscle function in mdx mice with a noticeable reduction in their serum creatine kinase values. Subcutaneous or oral administration of PTC124 to a mice expressing human CFTR-G542X transgene is shown to suppress G542X nonsense mutation and restore hCFTR function and cAMP-stimulated chloride channel activity (60 mg/kg for 3 weeks). Also acts as a potent and reversible inhibitor of purified firefly luciferase (FLuc; IC50 ~ 10 nM) and protects it from tryptic degradation, but does not affect Renilla reniformis luciferase (RLuc).
          Catalogue Number530918
          Brand Family Calbiochem®
          Synonyms3-(5-(2-Fluorophenyl)-1,2,4-oxadiazol-3-yl)benzoic acid, Premature Termination Codon Mutations Inhibitor, PTC124
          References
          ReferencesMcElroy, S.P., et al. 2013. PLoS Biol. 11, e1001593.
          Auld, D.S., et al. 2010. Proc. Natl. Acad. Sci. USA. 107, 4878.
          Auld, D.S., et al. 2009. Proc. Natl. Acad. Sci. USA. 106, 3585.
          Du, M., et al. 2008. Proc. Natl. Acad. Sci. USA. 105, 2064.
          Welch, E.M., et al. 2007. Nature. 447, 87.
          Product Information
          CAS number775304-57-9
          FormWhite solid
          Hill FormulaC₁₅H₉FN₂O₃
          Chemical formulaC₁₅H₉FN₂O₃
          ReversibleY
          Applications
          Biological Information
          Primary TargetPTC
          Secondary targetfirefly luciferase
          Purity≥95% by HPLC
          Physicochemical Information
          Cell permeableY
          Dimensions
          Materials Information
          Toxicological Information
          Safety Information according to GHS
          Safety Information
          Product Usage Statements
          Storage and Shipping Information
          Ship Code Ambient Temperature Only
          Toxicity Standard Handling
          Storage +2°C to +8°C
          Protect from Light Protect from light
          Do not freeze Ok to freeze
          Special InstructionsFollowing reconstitution, aliquot and freeze (-20°C). Stock solutions are stable for up to 6 months at -20°C.
          Packaging Information
          Packaged under inert gas Packaged under inert gas
          Transport Information
          Supplemental Information
          Specifications

          Documentation

          (M)SDS

          タイトル

          英語版製品安全データシート((M)SDS) 

          参考資料

          参考資料の概要
          McElroy, S.P., et al. 2013. PLoS Biol. 11, e1001593.
          Auld, D.S., et al. 2010. Proc. Natl. Acad. Sci. USA. 107, 4878.
          Auld, D.S., et al. 2009. Proc. Natl. Acad. Sci. USA. 106, 3585.
          Du, M., et al. 2008. Proc. Natl. Acad. Sci. USA. 105, 2064.
          Welch, E.M., et al. 2007. Nature. 447, 87.

          カタログ

          タイトル
          New Products Flyer, Volume 2, 2015, Immunology Feature

          技術情報

          タイトル
          White Paper: Further considerations of antibody validation and usage.
          データシート

          Note that this data sheet is not lot-specific and is representative of the current specifications for this product. Please consult the vial label and the certificate of analysis for information on specific lots. Also note that shipping conditions may differ from storage conditions.

          Revision05-January-2015 JSW
          Synonyms3-(5-(2-Fluorophenyl)-1,2,4-oxadiazol-3-yl)benzoic acid, Premature Termination Codon Mutations Inhibitor, PTC124
          DescriptionA cell-permeable, non-toxic, orally bioavailable oxadiazole compound that is reported to promote read-through of premature, but not normal, translation termination codons and thereby suppress premature termination in protein synthesis caused by nonsense mutations (in ~10 to 100 nM with maximal activity reported at 3 µM). Promotes the suppression of nonsense alleles of the dystrophin gene in primary human muscle cells and in a mouse model of Duchenne muscular dystrophy (mdx). Also reported to rescue striated muscle function in mdx mice with a noticeable reduction in their serum creatine kinase values. Subcutaneous or oral administration of PTC124 to a mice expressing human CFTR-G542X transgene is shown to suppress G542X nonsense mutation and restore hCFTR function and cAMP-stimulated chloride channel activity (60 mg/kg for 3 weeks). Also acts as a potent and reversible inhibitor of purified firefly luciferase (FLuc; IC50 ~ 10 nM) and protects it from tryptic degradation, but does not affect Renilla reniformis luciferase (RLuc).
          FormWhite solid
          Intert gas (Yes/No) Packaged under inert gas
          CAS number775304-57-9
          Chemical formulaC₁₅H₉FN₂O₃
          Purity≥95% by HPLC
          SolubilityDMSO (50 mg/ml)
          Storage +2°C to +8°C
          Protect from light
          Do Not Freeze Ok to freeze
          Special InstructionsFollowing reconstitution, aliquot and freeze (-20°C). Stock solutions are stable for up to 6 months at -20°C.
          Toxicity Standard Handling
          ReferencesMcElroy, S.P., et al. 2013. PLoS Biol. 11, e1001593.
          Auld, D.S., et al. 2010. Proc. Natl. Acad. Sci. USA. 107, 4878.
          Auld, D.S., et al. 2009. Proc. Natl. Acad. Sci. USA. 106, 3585.
          Du, M., et al. 2008. Proc. Natl. Acad. Sci. USA. 105, 2064.
          Welch, E.M., et al. 2007. Nature. 447, 87.