Anti-Dystrophin Antibody, clone 2C6 (MANDYS106), Alexa Fluor^™ 488 Conjugate

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), 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.

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 (Anthony, K., et al. (2011). Brain. 134(Pt 12):3547-3559).

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

Research Category
Cell Structure

The unconjugated antibody (Cat. No. MABT827) and Alexa Fluor^™ 555 conjugate (Cat. No. MABT827-AF555) are also available for Immunohistochemistry, Immunofluorescence, and Western Blotting applications.

This mouse monoclonal Anti-Dystrophin Antibody, clone 2C6 (MANDYS106), Alexa Fluor^™ 488 Conjugate, Cat. No. MABT827-AF488 is validated for use in Immunofluorescence for the detection of Dystrophin.

Evaluated by Immunofluorescence in human skeletal muscle tissue sections.

Immunofluorescence Analysis: A 1:25 dilution of this antibody detected dystrophin in human skeletal muscle tissue sections.

~427 kDa observed.

Protein G purified.

Purified mouse IgG2a Alexa Fluor^™ 488 conjugate in buffer containing PBS, 1.5% BSA with 0.05% sodium azide

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

Concentration: Please refer to lot specific datasheet.

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