title: Functional significance of dystrophin-positive fibers in Duchenne and Becker muscular dystrophy.
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.
authors.affiliation: Department of Pediatrics, Atutürk University Faculty of Medicine, Erzurum, Turkey.
authors.forename: H A
authors.initials: HA
authors.lastname: Taşdemir
authors.forename: E
authors.initials: E
authors.lastname: Kotiloğlu
authors.forename: H
authors.initials: H
authors.lastname: Topaloğlu
authors.forename: G
authors.initials: G
authors.lastname: Kale
authors.forename: D P
authors.initials: DP
authors.lastname: Dinçer
authors.forename: K
authors.initials: K
authors.lastname: Yalaz
authors.forename: Y
authors.initials: Y
authors.lastname: Renda
mesh_terms: D016189:Dystrophin / Q000032:analysis* / Q000502:physiology*; D006801:Humans; D007150:Immunohistochemistry; D018485:Muscle Fibers, Skeletal / Q000276:immunology* / Q000378:metabolism*; D009136:Muscular Dystrophies / Q000276:immunology* / Q000378:metabolism*
chemical_list: D016189:Dystrophin