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\nCellular Localization: Cell Membrane\n\nPositive Control: Skeletal muscle.\n\nApplications: \nSuitable for use in Immunofluorescence and Immunohistochemistry (paraffin-embedded sections). Other applications not tested.\n\nRecommended Dilution:\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, aliquot 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

D9910-02B

Size

500ul

Applications

IF, IHC

Hosts

Mouse

Reactivities

Hum, Mouse, Rat, Ch/Bird, Xen/Amph

Form

10mM PBS, pH7.4, 0.2%BSA

P Type

Mab

Purity

Mouse ascites

Isotype

IgG1

References

This product has been used in: Sealock R et al. Localization of dystrophin relative to acetylcholine receptor domains in electric tissue and adult and cultured skeletal muscle. J Cell Biol 113:1133-44 (1991). <PUBMED:2040646>

Additional Info

Highly specific to dystrophin and shows no crossreaction with C-protein (an isoform of &#225;-actinin), &#225;-actin, or human muscle spectrin. Species Crossreactivity: Crossreacts with Human, Chicken, Mouse, Rat, Xenopus laevis and Torpedo ray.