MABT827

Anti-Dystrophin Antibody, clone 2C6 (MANDYS106)

clone 2C6 (MANDYS106), from mouse

• Sinónimos: Dystrophin
• UNSPSC Code: 12352203
• eCl@ss: 32160702
• NACRES: NA.41

Propiedades

• biological source: mouse
• Quality Level: 100
• antibody form: purified antibody
• antibody product type: primary antibodies
• clone: 2C6 (MANDYS106), monoclonal
• species reactivity: human
• technique(s): immunofluorescence: suitable; immunohistochemistry: suitable; western blot: suitable
• isotype: IgG2aκ
• NCBI accession no.: NP_000100
• UniProt accession no.: P11532-1
• shipped in: wet ice
• target post-translational modification: unmodified
• Gene Information: human ... DMD(1756)

Descripción

General description

Dystrophin (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) and plays an important role in stabilizing the muscle fiber 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 is designed to transform the DMD phenotype to that of the milder disorder, Becker muscular dystrophy (BMD), which is typically caused by in-frame dystrophin deletions that allow the production of an internally deleted, but partially functional dystrophin.

Specificity

Detects 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.

Immunogen

Epitope: Exon 43-coded pectrin-like repeat 16 region

TrpE-tagged recombinant protein corresponding to the Exon 43-coded pectrin-like repeat 16 region of human Dystrophin.

Application

Anti-Dystrophin Antibody, clone 2C6 (MANDYS106) is an antibody against Dystrophin for use in Immunohistochemistry, Immunofluorescence, Western Blotting.

Immunohistochemistry Analysis: A represenative lot stained sarcolemma of muscle fiber cells in tissue samples from healthy donors, while much reduced staining was observed in biopsy samples from patients with Becker muscular dystrophy (BMD) and no staining is seen with Duchenne muscular dystrophy (DMD) biopsy samples (Anthony, K., et al. (2011). Brain. 134(Pt 12):3547-3559).
Immunofluorescence Analysis: A represenative lot was employed together with a spectrin antibody in dual immunofluorescent sarcolemma staining for assessing dystrophin levels of muscle fiber cells in muscle biopsies from healthy donors and Becker muscular dystrophy (BMD) patients (Beekman, C., et al. (2014). PLoS One. 9(9):e107494).

Research Category
Cell Structure

Research Sub Category
Adhesion (CAMs)

Quality

Evaluated by Immunohistochemistry in human skeletal muscle myocytes.

Immunohistochemistry Analysis: A 1:50 dilution of this antibody detected Dystrophin in human skeletal muscle myocytes.

Target description

~427 kDa observed

Physical form

Format: Purified

Protein G Purified

Purified mouse monoclonal IgG2aκ antibody in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.

Storage and Stability

Stable for 1 year at 2-8°C from date of receipt.

Other Notes

Concentration: Please refer to lot specific datasheet.

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
• flash_point_f: Not applicable
• flash_point_c: Not applicable