MABT1544

Anti-Nebulin exon 144 Antibody, clone NEB-9H1

• Sinónimos: NEB;NEM2
• UNSPSC Code: 12352203

Propiedades

• biological source: mouse
• Quality Level: 200
• antibody form: purified antibody
• antibody product type: primary antibodies
• clone: NEB-9H1, monoclonal
• mol wt: calculated mol wt 772.937 kDa; observed mol wt ~47 kDa
• purified by: using protein G
• species reactivity: human
• packaging: antibody small pack of 100 μL
• technique(s): immunofluorescence: suitable; immunohistochemistry: suitable; western blot: suitable
• isotype: IgG1κ
• Protein ID accession no.: NP_004534
• UniProt accession no.: P20929
• storage temp.: 2-8°C
• target post-translational modification: unmodified
• Gene Information: human ... NEB(4703)

Descripción

General description

Nebulin (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).

Specificity

Clone 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).

Immunogen

GST-tagged recombinant fragment corresponding to amino acids encoded by exons 138-144 expressed in E.coli.

Application

Qualtiy Control Testing

Evaluated 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.

Tested 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

Physical form

Purified mouse monoclonal antibody IgG1 in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.

Reconstitution

0.5 mg/mL. Please refer to guidance on suggested starting dilutions and/or titers per application and sample type.

Storage and Stability

Recommended storage: +2°C to +8°C.

Other Notes

Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration.

Disclaimer

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.

Información de seguridad

• Storage Class: 12 - Non Combustible Liquids
• wgk_germany: WGK 1