Dystrophin, NT (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. Not suitable for Immunohistochemistry paraffin sections. Other applications not tested.\n\nRecommended Dilution:\nWestern Blot: 1:25-1:50\nImmunohistochemistry (Frozen unfixed): Neat-1:20 for 1 hr at RT. \nOptimal dilutions to be determined by the researcher.\n\nPositive Control: \nWestern Blot: Skeletal muscle, doublet of bands at ~400kD.\nImmunohistochemistry: For reliable interpretation of dystrophin staining results, a spectrin control must be used. \nSnap frozen normal human striated muscle (staining pattern continuious 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

D9910-20A

Size

2.5ml

Applications

IHC, WB

Hosts

Mouse

Reactivities

Hum, NHP

Form

Supplied as a lyophilized powder from PBS, 1% BSA, 15mM sodium azide. Reconstitute with 2.5ml sterile ddH2O.

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).

Additional Info

Recognizes Dystrophin. Epitope: binds to regions from aa 308-351. These residues span the junction of exons 9 and 10. The epitope recognized may be part of the hinge region between the amino acid domain and the central rod domain. Species Crossreactivity: human and monkey.