Anti-Dystrophin (DMD) (Marker of Duchenne and Becker Muscular Dystrophy) Monoclonal Antibody(Clone: DMD/3242)
Fig. 1: Formalin-fixed, paraffin-embedded human Skeletal Muscle stained with Dystrophin Monospecific Mouse Monoclonal Antibody (DMD/3242).
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| Amount : | 100 µg |
| Isotype : | Mouse IgG2b, kappa |
| Content : | 200 µg/ml of Ab Purified from Bioreactor Concentrate by Protein A/G. Prepared in 10mM PBS with 0.05% BSA & 0.05% azide. Also available WITHOUT BSA & azide at 1.0mg/ml. |
| Storage condition : | Antibody with azide - store at 2 to 8°C. Antibody without azide - store at -20 to -80°C. Antibody is stable for 24 months. Non-hazardous. |
Dystrophin-glycoprotein complex (DGC) connects the F-Actin cytoskeleton on the inner surface of muscle fibers to the surrounding extracellular matrix, throµgh the cell membrane interface. A deficiency in this protein contributes to Duchenne (DMD) and Becker (BMD) muscular dystrophies. The human dystrophin gene measures 2.4 megabases, has more than 80 exons, produces a 14 kb mRNA and contains at least 8 independent tissue-specific promoters and 2 poly A sites. The dystrophin mRNA can undergo differential splicing and produce a range of transcripts that encode a large set of proteins. Dystrophin represents approximately 0.002% of total striated muscle protein and localizes to triadic junctions in skeletal muscle, where it is thoµght to influence calcium ion homeostasis and force transmission.
ELISA (For coating, order Ab without BSA);,Immunohistochemistry (Formalin-fixed) (1-2ug/ml for 30 minutes at RT),(Staining of formalin-fixed tissues requires heating tissue sections in 10mM Tris with 1mM EDTA, pH 9.0, for 45 min at 95°C followed by cooling at RT for 20 minutes);
For Research Use Only. Not for use in diagnostic/therapeutics procedures.
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