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Key Specifications Table
| Species Reactivity | Key Applications | Host | Format | Antibody Type |
|---|---|---|---|---|
| H | IHC, IF, WB | M | Purified | Monoclonal Antibody |
| Description | |
|---|---|
| Catalogue Number | MABT827 |
| Description | Anti-Dystrophin Antibody, clone 2C6 (MANDYS106) |
| Alternate Names |
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| Background Information | 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. |
| Product Information | |
|---|---|
| Format | Purified |
| Presentation | Purified mouse monoclonal IgG2aκ antibody in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide. |
| Quality Level | MQ100 |
| Applications | |
|---|---|
| Application | Anti-Dystrophin Antibody, clone 2C6 (MANDYS106) is an antibody against Dystrophin for use in Immunohistochemistry, Immunofluorescence, Western Blotting. |
| Key Applications |
|
| Application Notes | 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). |
| Biological Information | |
|---|---|
| Immunogen | TrpE-tagged recombinant protein corresponding to the Exon 43-coded pectrin-like repeat 16 region of human Dystrophin. |
| Epitope | Exon 43-coded pectrin-like repeat 16 region |
| Clone | 2C6 (MANDYS106) |
| Concentration | Please refer to lot specific datasheet. |
| Host | Mouse |
| 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. |
| Isotype | IgG2aκ |
| Species Reactivity |
|
| Antibody Type | Monoclonal Antibody |
| Entrez Gene Number |
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| Gene Symbol |
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| Purification Method | Protein G Purified |
| UniProt Number |
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| Molecular Weight | ~427 kDa observed |
| Product Usage Statements | |
|---|---|
| Quality Assurance | Evaluated by Immunohistochemistry in human skeletal muscle myocytes. Immunohistochemistry Analysis: A 1:50 dilution of this antibody detected Dystrophin in human skeletal muscle myocytes. |
| Usage Statement |
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| Storage and Shipping Information | |
|---|---|
| Storage Conditions | Stable for 1 year at 2-8°C from date of receipt. |
| Packaging Information | |
|---|---|
| Material Size | 100 µL |