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MilliporeSigma™ Mouse anti-Dystrophin, Clone: 2C6 (MANDYS106),

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MABT827MI 100 μL
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Description

Mouse Monoclonal Antibody

Specifically detects Dystrophin clone: 2C6 (MANDYS106) in Human samples, and it is validated for Western Blotting

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.
TRUSTED_SUSTAINABILITY

Specifications

Antigen Dystrophin
Applications Western Blot
Classification Monoclonal
Clone 2C6 (MANDYS106)
Formulation Purified mouse monoclonal IgG2aκ antibody in buffer containing 0.1M Tris-Glycine (pH 7.4), 150mM NaCl with 0.05% sodium azide.
Gene Accession No. P11532-1
Gene Symbols DMD
Host Species Mouse
Immunogen TrpE-tagged recombinant protein corresponding to the Exon 43-coded pectrin-like repeat 16 region of human Dystrophin.
Purification Method Protein G Purified
Quantity 100 μL
Regulatory Status RUO
Research Discipline Cell Structure
Primary or Secondary Primary
Gene ID (Entrez) NP_000100
Target Species Human
Content And Storage Stable for one year at 2°C to 8°C from date of receipt.
Form Purified
Isotype IgG2a κ
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For Research Use Only

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