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Original Article

Functional Significance of Dystrophin-Positive Fibers in Duchenne and Becker Muscular Dystrophy

, , , , , & show all
Pages 583-590 | Received 29 Mar 1995, Accepted 15 Aug 1995, Published online: 16 Apr 2010
 

Abstract

In this study, the ratios of dystrophin-positive (+), partially deficient (±), and deficient (-) fibers were investigated immunohistochemically in 28 Duchenne muscular dystrophy (DMD) and 4 Becker muscular dystrophy (BMD) patients using Dys I (midrod), Dys II (COOH-terminal), and Dys III (NH2-terminal) antibodies. In the biopsies of DMD patients, Dys II was negative in all cases; the mean ratio of Dys 1 (+) fibers was 0.05%, Dys I (±) 1.02%, Dys III (+) 0.27%, and Dys III (±) 0.75%. There was no correlation between these (+) or (±) fibers and the severity of clinical or laboratory findings. In BMD patients, it was shown that amino and carboxyl terminals of dystrophin could be affected in addition to the midportion.

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