Dystrophin, Rod Domain (Duchenne Muscular Dystrophy Protein, DMD, BMD, CMD3B, DXS142, DXS164, DXS206, DXS230, DXS239, DXS268, DXS269, DXS270, DXS272, Muscular Dystrophy Duchenne and Becker Types)

Dystrophin is a rod-like cytoskeletal protein found adja- cent to the muscle membrane. It is one component of a complex system that links actin on the inside of muscle fibers to extracellular matrix proteins that surround the fibers. Mutations in the genes that encode for dys- trophin lead to altered expression of this protein and cause muscular dystrophy. This antibody will help detect dystrophin in normal tissue sections.\n\nApplications: \nSuitable for use in Western Blot and Immunohistochemistry. Other applications not tested.\n\nRecommended Dilution:\nWestern Blot: 1:100-1:250\nImmunohistochemistry (Frozen unfixed): 1:20 for 1 hr. at RT (neat)\nOptimal dilutions to be determined by the researcher.\n\nPositive Control: \nWestern Blot: Skeletal muscle, strong doublet of bands at ~400kD and lower molecular weight metabolites.\nImmunohistochemistry: Snap frozen normal human or rat striated muscle. Staining pattern-continuous rim of staining at the periphery of muscle fibers.\n\nStorage and Stability:\nLyophilized powder may be stored at -20 degrees C. Stable for 12 months at -20 degrees C. Reconstitute with sterile ddH2O. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Reconstituted product is stable for 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

D9910-30A

Size

2.5ml

Applications

IHC, WB

Hosts

Mouse

Reactivities

Hum, Can, Prc, Rabbit

Form

Supplied as a lyophilized powder in PBS, 1% BSA, 15mM sodium azide. Reconstitute with 2.5ml dH2O.

P Type

Mab

Purity

Supernatant

Isotype

IgG2a

References

1.Sheriffs I N, Rampling D and Smith V V. Paraffin wax embedded muscle is suitable for the diagnosis of muscular dystrophy. Journal of Clinical Pathology. 54:517-520 (2001). 2.Hoffman E P, Brown R H Jr and Kunkell L M. Dystrophin the protein of the Duchenne muscular dystrophy locus. Cell. 51(6):919-928 (1987).

Additional Info

Recognizes Dystrophin. Species Crossreactivity: Canine, hamster, human, porcine, rabbit and rat.