Technical Data
D9910-30
Dystrophin, Rod Domain
Description:
Dystrophin is a member of the spectrin/alpha-actinin family of actin-binding, triple helix rod-containing proteins. It is absent or greatly reduced in individuals with the X-linked recessive Duchennes muscular dystrophy disorder, as well as in mice with the mdx (murine muscular dystrophy) mutation.

Applications:
Suitable for use in Western Blot, Immunohistochemistry and EM Gold. Other applications not tested.

Recommended Dilution:
Western Blot: 1:100-1:250
Immunohistochemistry(Fresh frozen, unfixed tissue): Neat 1:20
Not recommended for use on paraffin embedded tissue.
EM Gold: Undiluted 90 minute incubation at 25C. Light fixation with 2% formaldehyde + 0.001% glutaraldehyde for 1 hour. Use 2.3M sucrose as cryoprotectant.
Optimal dilutions to be determined by the researcher.

Expected Staining Pattern:
Light microscopy: Continuous rim of labeling at the periphery of muscle fibers
Western Blot: Strong double bands at ~400kD and metabolites of lower molecular mass
E.M. Gold: Close to the cytoplasmic face of the plasma membrane.

Positive Control:
Snap frozen normal human or rat striated muscle.

Storage and Stability:
May be stored at 4C for short-term only. For long-term storage and to avoid repeated freezing and thawing, add sterile 40-50% glycerol, aliquot and store at -20C. Aliquots are stable for at least 12 months at -20C. 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.
TypeIsotypeCloneGrade
MabIgG2a2Q1101Supernatant
SizeStorageShippingSourceHost
1ml4C (-20C Glycerol)Blue IceHumanMouse
Concentration:
Not Determined
Immunogen:
Bacterial fusion protein
Purity:
Supernatant
Form
Supplied as a liquid in PBS, 1% BSA, 15mM sodium azide.
Specificity:
Recognizes mid rod domain of human dystrophin between aa1181-1388. Reacts with skeletal, cardiac and smooth muscle dystrophin. Reacts on blots with the brain isoform. No reactivity with mdx mouse tissue or DMD/BMD patients who have a gene deletion. Does not react with chicken dystrophin. Species Crossreactivity: mouse, rat, rabbit and canine
Intended for research use only. Not for use in human, therapeutic, or diagnostic applications.
1. Cullen, M.J. et al., Proceedings of the Royal Society (1990) 240:197210. 2. England, S.B., et al., Nature (1990) 324:180182. 3. Nicholson, L.V.B., et al., Journal of Neurological Sciences (1989) 94:125136. 4. Nicholson, L.V.B., et al., Journal of Neurological Sciences (1989) 94:137146. 5. Nicholson, L.V.B., et al., Acta Neuropathologica (1990) 80:239250. 6. Nordenskjold, M., et al., Human Genetics (1990) 84:207209. 7. Norman, A.M., et al., Archives of Disease in Childhood (1989) 64:15011503. 8. Nicholson, L.V.B. et al., Acta. Neurol. Scand.(1992) 86:814. 9. Voit, T. et al., Neuropediatrics (1991) 22:152162. 10. Slater, C.R., et al. Journal of Neurological Sciences (1991) 101:187192.