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MABT1544-100UL Anti-Nebulin exon 144 Antibody, clone NEB-9H1

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MABT1544-100UL
100 µL  
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      Overview

      Replacement Information
      Description
      Catalogue NumberMABT1544-100UL
      DescriptionAnti-Nebulin exon 144 Antibody, clone NEB-9H1
      Alternate Names
      • NEB
      • NEM2
      Background InformationNebulin (UniProt: P20929; also known as NEB, NEM2) is encoded by the NEB gene (Gene ID: 4703) in human. Nebulin is a highly conserved, muscle-specific protein that is located in the thin filament of striated muscle. It is involved in maintaining the structural integrity of sarcomeres and the membrane system associated with the myofibrils. It is shown to bind and stabilize F-actin. Nebulin has a highly repetitive protein structure with about 97% of the protein consisting of modules of 30 to 35 amino-acids arranged into simple repeats or super repeats. Each repeat binds to actin along the length of the thin filament. The repeat modules contain conserved SDxxYK consensus sequence that binds F-actin. The N-terminal and the C-terminal ends of Nebulin contain unique protein domains. The C-terminal is anchored in the Z-disc of the muscle sarcomere and contains a conserved Src homology (SH3) domain. Although all known exons in the NEB gene can encode a protein with up to 8,525 amino-acids, alternative splicing may produce somewhat smaller proteins. Most of these alternative splicing events simply remove exons from the nebulin mRNAs, however, all nebulin mRNAs contain either exon 143 or exon 144, but never both. Exon 143 codes for amino acids 5242-5272 and exon 144 codes for amino acids 5277-5307. The sequence encoded by exon 143 shows complete homology between mouse, rat, and human. Studies on NEB knockout mice demonstrate a role for nebulin in establishing lateral connectivity of myofibrils and they lose their tight lateral register on stretch. (Ref.: Lam, L., et al. (2018). Sci. Rep. 8; 15728; Tonino, P., et al. (2010). J. Cell Sci. 123(3); 384-391; Donner, K., et al. (2004). Eur. J. Hum. Genet. 12, 744 751).
      References
      Product Information
      FormatPurified
      PresentationPurified mouse monoclonal antibody IgG1 in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
      Quality LevelMQ200
      Applications
      ApplicationAnti-Nebulin exon 144, clone NEB-9H1, Cat. No. MABT1544, is a mouse monoclonal antibody that detects Nebulin and is tested for use in Immunofluorescence, Immunohistochemistry, and Western Blotting.
      Key Applications
      • Western Blotting
      • Immunofluorescence
      • Immunohistochemistry
      Application NotesTested applications

      Immunohistochemistry Applications: A representative lot detected Nebulin exon 144 in Immunohistochemistry applications (Lam, L.T., et al. (2018). Sci Rep. 8(1):15728).

      Western Blotting Analysis: A representative lot detected Nebulin exon 144 in Western Blotting applications (Lam, L.T., et al. (2018). Sci Rep. 8(1):15728).

      Immunofluorescence Analysis: A representative lot detected Nebulin exon 144 in Immunofluorescence applications (Lam, L.T., et al. (2018). Sci Rep. 8(1):15728).

      Note: Actual optimal working dilutions must be determined by end user as specimens, and experimental conditions may vary with the end user
      Biological Information
      ImmunogenGST-tagged recombinant fragment corresponding to amino acids encoded by exons 138-144 expressed in E.coli.
      EpitopeC-terminal half
      CloneNEB-9H1
      Concentration0.5 mg/mL. Please refer to guidance on suggested starting dilutions and/or titers per application and sample type.
      HostMouse
      SpecificityClone NEB-9H1 is a mouse monoclonal antibody that detects Nebulin. It targets an epitope with in the sequence encoded by exon 144 (aa 5277-5307).
      IsotypeIgG1κ
      Species Reactivity
      • Human
      Species Reactivity NoteHuman.
      Antibody TypeMonoclonal Antibody
      Entrez Gene Number
      Gene Symbol
      • NEB
      Purification MethodProtein G purified
      UniProt Number
      Molecular Weight~47 kDa observed; 772.9, 986.66, 986.68, and 772.937 kDa calculated, respectively for isoforms 1, 2, 3 and 4. Uncharacterized bands may be observed in some lysate(s).
      Physicochemical Information
      Dimensions
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality AssuranceEvaluated by Western Blotting in Nebulin recombinant fragments.

      Western Blotting Analysis (WB): A 1:1,000 dilution of this antibody detected recombinant Nebulin fragments encoded by exons 138-144, but not by exons 138-143.
      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 ConditionsRecommended storage: +2°C to +8°C.
      Packaging Information
      Material Size100 µL
      Transport Information
      Supplemental Information
      Specifications
      Global Trade Item Number
      Catalogue Number GTIN
      MABT1544-100UL 04065268978504