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MABT827-AF555 Anti-Dystrophin Antibody, clone 2C6 (MANDYS106), Alexa Fluor® 555 Conjugate

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MABT827-AF555
100 µL  
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      Overview

      Replacement Information

      Key Spec Table

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      HIFMAlexaFluor®555Monoclonal Antibody
      Description
      Catalogue NumberMABT827-AF555
      DescriptionAnti-Dystrophin Antibody, clone 2C6 (MANDYS106), Alexa Fluor® 555 Conjugate
      Alternate Names
      • Dystrophin
      Background InformationDystrophin (UniProt P11532) is encoded by the DMD (also known as BMD, CMD3B, DXS142, DXS164, DXS206, DXS230, DXS239, DXS268, DXS269, DXS270, DXS272, MRX85) gene (Gene ID 1756) in human. Dystrophin is localized to the inner part of the muscle fiber cell membrane (sarcolemma), where it forms the dystrophin-associated glycoprotein complex (DGC) that links the extracellular matrix to the actin cytoskeleton. Dystrophin plays an important role in stabilizing the muscle fibre against the mechanical forces of muscle contraction by providing a shock-absorbing connection between the cytoskeleton and the extracellular matrix. Duchenne muscular dystrophy (DMD) is caused by gene mutations that disrupt the open reading frame (ORF) and prevent the full translation of dystrophin. ORF restoration by exon skipping using antisense oligonucleotides targeted to splicing elements are designed to transform the DMD phenotype to that of the milder disorder, Becker muscular dystrophy (BMD), typically caused by in-frame dystrophin deletions that allow the production of an internally deleted but partially functional dystrophin.
      References
      Product Information
      FormatAlexaFluor®555
      Control
      • The unconjugated antibody (Cat. No. MABT827) and Alexa Fluor® 488 conjugate (Cat. No. MABT827-AF488) are also available for Immunohistochemistry
      • Immunofluorescence
      • and Western Blotting applications.
      PresentationPurified mouse IgG2a Alexa Fluor® 555 conjugate in buffer containing PBS, 1.5% BSA with 0.05% sodium azide
      Quality LevelMQ100
      Applications
      ApplicationThis mouse monoclonal Anti-Dystrophin Antibody, clone 2C6 (MANDYS106), Alexa Fluor® 555 Conjugate, Cat. No. MABT827-AF555 is validated for use in Immunofluorescence for the detection of Dystrophin.
      Key Applications
      • Immunofluorescence
      Application NotesThe unconjugated antibody (Cat. No. MABT827) and Alexa Fluor® 488 conjugate (Cat. No. MABT827-AF488) are also available for Immunohistochemistry, Immunofluorescence, and Western Blotting applications.
      Biological Information
      ImmunogenTrpE-tagged recombinant protein corresponding to the Exon 43-coded pectrin-like repeat 16 region of human Dystrophin.
      Clone2C6 (MANDYS106)
      ConcentrationPlease refer to lot specific datasheet.
      HostMouse
      SpecificityDetects dystrophin spliced isoforms 1-4, but not isoforms 5-10, or utrophin. Positive muscle membrane staining of tissue samples from healthy donors, reduced staining of Becker muscular dystrophy (BMD) biopsies, and no staining is seen with Duchenne muscular dystrophy (DMD) biopsy samples (Anthony, K., et al. (2011). Brain. 134(Pt 12):3547-3559).
      IsotypeIgG2aκ
      Species Reactivity
      • Human
      Species Reactivity NoteHuman.
      Antibody TypeMonoclonal Antibody
      Entrez Gene Number
      Gene Symbol
      • DMD
      • BMD
      • CMD3B
      • DXS142
      • DXS164
      • DXS206
      • DXS230
      • DXS239
      • DXS268
      • DXS269
      • DXS270
      • DXS272
      • MRX85
      Purification MethodProtein G purified.
      UniProt Number
      Molecular Weight~427 kDa observed.
      Physicochemical Information
      Dimensions
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality AssuranceEvaluated by Immunofluorescence in human skeletal muscle tissue sections.

      Immunofluorescence Analysis: A 1:100 dilution of this antibody detected dystrophin in human skeletal muscle tissue sections.
      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 µL
      Transport Information
      Supplemental Information
      Specifications
      Global Trade Item Number
      Catalogue Number GTIN
      MABT827-AF555 04054839081453

      Documentation

      Anti-Dystrophin Antibody, clone 2C6 (MANDYS106), Alexa Fluor® 555 Conjugate SDS

      Title

      Safety Data Sheet (SDS) 

      Anti-Dystrophin Antibody, clone 2C6 (MANDYS106), Alexa Fluor® 555 Conjugate Certificates of Analysis

      TitleLot Number
      Anti-Dystrophin, clone 2C6 (MANDYS106), Alexa Fluor® 555 Conjugate - 3230942 3230942
      Anti-Dystrophin, clone 2C6 (MANDYS106), Alexa Fluor® 555 Conjugate - 3460517 3460517
      Anti-Dystrophin, clone 2C6 (MANDYS106), Alexa Fluor® 555 Conjugate - 3673687 3673687
      Anti-Dystrophin, clone 2C6 (MANDYS106), Alexa Fluor® 555 Conjugate - 3821559 3821559
      Anti-Dystrophin, clone 2C6 (MANDYS106), Alexa Fluor® 555 Conjugate - 4076676 4076676
      Anti-Dystrophin, clone 2C6 (MANDYS106), Alexa Fluor® 555 Conjugate - 4078199 4078199
      Anti-Dystrophin, clone 2C6 (MANDYS106), Alexa Fluor® 555 Conjugate - 4193542 4193542
      Anti-Dystrophin, clone 2C6 (MANDYS106), Alexa Fluor® 555 Conjugate -Q2767846 Q2767846