Functional significance of dystrophin-positive fibers in Duchenne and Becker muscular dystrophy.
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.
Taşdemir
H A
HA
Department of Pediatrics, Atutürk University Faculty of Medicine, Erzurum, Turkey.
Kotiloğlu
E
E
Topaloğlu
H
H
Kale
G
G
Dinçer
D P
DP
Yalaz
K
K
Renda
Y
Y
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*
D016189:Dystrophin