Asymptomatic Becker muscular dystrophy: expression of dystrophin and dystrophin-related protein.
The expression of dystrophin and a dystrophin-related protein in muscle from 3 asymptomatic Becker muscular dystrophy patients is reported. Histology demonstrated moderate variation in fiber size with clustered basophilic regenerating fibers. Immunostaining for dystrophin revealed a patchy appearance on most fibers, but never on clustered regenerating fibers. Those regenerating fibers did, however, have positive immunoreactivity to a dystrophin-related protein. It is speculated that overexpression of the dystrophin-related protein in regenerating muscle fibers may contribute to the slow progression of muscle weakness or atrophy.
Tachi
N
N
Department of Pediatrics, Sapporo Medical College, Japan.
Wakai
S
S
Watanabe
Y
Y
Ohya
K
K
Chiba
S
S
D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D003598:Cytoskeletal Proteins / Q000032:analysis / Q000235:genetics*; D016189:Dystrophin / Q000032:analysis / Q000235:genetics*; D005786:Gene Expression Regulation / Q000502:physiology; D006801:Humans; D007124:Immunoenzyme Techniques; D008297:Male; D008565:Membrane Proteins; D009132:Muscles / Q000473:pathology; D009136:Muscular Dystrophies / Q000235:genetics* / Q000473:pathology; D049411:Utrophin
D003598:Cytoskeletal Proteins; D016189:Dystrophin; D008565:Membrane Proteins; D049411:Utrophin