Delayed expression of dystrophin on regenerating muscle from two siblings with Becker muscular dystrophy.
We present here a unique expression of dystrophin on biopsied muscle from 2 siblings with Becker muscular dystrophy (BMD). They had neither muscle weakness nor atrophy. Clustered dystrophin-deficient fibers were constituted to regenerating basophilic fibers (mainly type 2C fiber) based on histochemical stainings. We speculate that the developmental delay in the expression of dystrophin is a characteristic finding in regenerating fibers from asymptomatic and young BMD patients, such as the siblings in this report.
Tachi
N
N
Department of Pediatrics, Sapporo Medical College, Japan.
Wakai
S
S
Watanabe
Y
Y
Chiba
S
S
Nagaoka
M
M
Minami
R
R
D002648:Child; D002675:Child, Preschool; D016189:Dystrophin / Q000032:analysis / Q000378:metabolism*; D006801:Humans; D015151:Immunoblotting; D007124:Immunoenzyme Techniques; D008297:Male; D009132:Muscles / Q000378:metabolism* / Q000473:pathology; D009136:Muscular Dystrophies / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D009218:Myosins / Q000032:analysis
D016189:Dystrophin; D009218:Myosins