Dystrophin

Dystrophin is a muscle membrane protein (427kD) which is absent, reduced or altered as a result of mutation in Duchenne and Becker muscular dystrophies (DMD/BMD) or its homologue in the mouse.8 Severe DMD is associated with a marked dystrophin deficiency whereas patients with the milder form of BMD show less pronounced abnormalities of protein expression. Because abnormalities in the protein expression occur specifically in patients with these types of muscular dystrophy, dystrophin analysis may be used to distinguish these conditions from other neuromuscular diseases. Predictions from the sequence suggest a structural protein on the inner face of the membrane, consisting of a 25-repeat, rod-like triple-helical domain separating an N-terminal actin-binding domain from two C-terminal domains, one of which is rich in cysteine.9 The large size of dystrophin and its low abundance (~0.01% of the total muscle protein) are a hindrance to the isolation of intact, native protein for structure/function studies. Monoclonal antibodies against defined regions10 of dystrophin provide a means for studying its structure and function, interactions with other proteins and the nature of the partial gene products produced in some patients carrying deletions in the dystrophin gene. The antibodies are useful in the prenatal or post-abortion diagnosis of muscular dystrophy carriers by immunohistological analyses.\n\nApplications: \nSuitable for use in Immunofluorescence, ELISA, Western Blot and Immunohistochemistry. Other applications not tested.\n\nRecommended Dilution:\nImmunohistochemistry: 1:100. Staining of fresh or frozen muscle tissue results in a clear labeling confined to the perifery (plasma membrane) of normal striated muscle fibers.\nOptimal dilutions to be determined by the researcher.\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. For long-term storage and to avoid repeated freezing and thawing, add sterile 40-50% glycerol, aliquot and store at -20 degrees C. Aliquots are stable for at least 12 months at -20 degrees C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

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SPECIFICATIONS

Catalog Number

D9910-02C

Size

100ul

Applications

ELISA, IF, IHC, WB

Hosts

Mouse

Reactivities

Hum, Mouse, Rat

Form

Supplied as a liquid, 0.1% sodium azide.

P Type

Mab

Purity

Ascites

Isotype

IgG1

References

1.Morris GE et al. J Immunol Methods 161:23-8 (1993). <PUBMED:8486926> 2. Nguyen TM et al. . Biochem J 288 (Pt 2):663-8 (1992). <PUBMED:1281410> 3. Hugnot JP et al. Proc Natl Acad Sci U S A 89:7506-10 (1992). <PUBMED:1380160> 4. Lederfein D et al. Proc Natl Acad Sci U S A 89:5346-50 (1992). <PUBMED:1319059> 5. Nguyen thi Man AJ et al. . FEBS Lett 262:237-40 (1990). <PUBMED:1692289> 6. Gussoni E et al. Nature 356:435-8 (1992). <PUBMED:1557125> 7. Ellis JM et al.. Lancet 336:881-2 (1990). <PUBMED:1699095>

Additional Info

Recognizes human dystrophin and does not react with alpha-actinin and utrophin, an autosomal homologue of dystrophin, also called dystrophin-related protein (DRP). In Western Blot stains the 70-75kD protein known as Apo-Dystrophin-1 or Dp71. This is detected in the brain as well as in lymphoblastoid cells, cultures of brain astroglial and neuronal cells, liver and Hep G2 cells (human hepatoma). Species Crossreactivity: fish, mouse and rat.