Clone Number: DY10/12B2
Immunogen: Fusion protein containing amino acids 67 to 713.
Concentration: n/a
Storage buffer: Lyophilized
Additional info: Dystrophin is the 427kD protein product of the DMD gene located on the X chromosome at position Xp21. Analyte Specific Reagent. Analytical and performance characteristics are not established. Product is a lyophilized tissue culture supernatant containing sodium azide as a preservative. The user is required to reconstitute the contents of the vial with the correct volume of sterile distilled water as indicated on the vial label. Reacts strongly with the amino terminal domain (between amino acids 321 and 494) of human dystrophin. Patient immunoreactivity indicates epitope is near exons 10 to 12. Epitope mapping suggests that sequences from amino acids 308 to 351 are involved in antibody binding. This region spans the junction of exons 9 and 10 and the epitope recognised may be part of a hinge region joining the amino domain to the central rod domain. No reactivity with DMD/BMD patients deleted for exons 10 to 12. No crossreaction is observed with mouse (high background only), rat, rabbit, dog, chicken, hamster and pig dystrophin.
References: Marafioti T et al. American Journal of Pathology. 162 (3): 861–871 (2003/Hess J et al. Molecular and Cellular Biology. 21 (5): 1531–1539 (2001)/Re D et al. Cancer Research. 61 (5): 2080–2084 (2001)
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