MABT1543

Anti-Nebulin Antibody, clone NEB-6B5

• Synonyme(s) : NEB;NEM2
• Code UNSPSC : 12352203

Propriétés

• Source biologique: mouse
• Niveau de qualité: 200
• Forme d'anticorps: purified antibody
• Type de produit anticorps: primary antibodies
• Clone: NEB-6B5, monoclonal
• Poids mol.: calculated mol wt 772.937 kDa; observed mol wt ~18 to 49 kDa
• Produit purifié par: affinity chromatography
• Espèces réactives: human
• Conditionnement: antibody small pack of 100 μg
• Technique(s): immunofluorescence: suitable; western blot: suitable
• Isotype: IgG1κ
• Séquence de l'épitope: C-terminal half
• Numéro d'accès Protein ID: NP_004534
• Numéro d'accès UniProt: P20929
• Température de stockage: 2-8°C
• Modification post-traductionnelle de la cible: unmodified
• Informations sur le gène: human ... NEB(4703)

Description

Description générale

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).

Spécificité

Clone NEB-6B5 is a mouse monoclonal antibody that detects Nebulin. It targets an epitope within the sequence encoded by exon 140 (aa 5137-5167).

Immunogène

GST-tagged recombinant fragments corresponding to amino acids encoded by exons 138-143 and 138-143 expressed in E.coli.

Application

Quality 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 138-143 and 138-144.

Tested applications

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

Western Blotting Analysis: A representative lot detected Nebulin in Western Blotting 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

Forme physique

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

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

Stockage et stabilité

Recommended storage: +2°C to +8°C.

Autres remarques

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

Clause de non-responsabilité

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.

Informations sur la sécurité

• Code de la classe de stockage: 12 - Non Combustible Liquids
• Classe de danger pour l'eau (WGK): WGK 1