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  • Title: Functional significance of dystrophin-positive fibers in Duchenne and Becker muscular dystrophy.
    Author: Taşdemir HA, Kotiloğlu E, Topaloğlu H, Kale G, Dinçer DP, Yalaz K, Renda Y.
    Journal: Pediatr Pathol Lab Med; 1996; 16(4):583-90. PubMed ID: 9025855.
    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 I (+) 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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