Dystrophin-related protein in Becker muscular dystrophy.
A benign Becker muscular dystrophy (BMD) patient with a marked decrease in dystrophin exhibited remarkable expression of dystrophin-related protein (DRP) on most of the muscle cell membrane. A phenotypic Duchenne muscular dystrophy patient with a truncated form of dystrophin exhibited no DRP expression on the muscle cell membrane except for the neuromuscular junction. Increased DRP expression might compensate for a lack of dystrophin in some BMD patients.
Higuchi
I
I
Third Department of Internal Medicine, Faculty of Medicine, Kagoshima University.
Niiyama
T
T
Fukunaga
H
H
Nakamura
K
K
Nakagawa
M
M
Osame
M
M
D000293:Adolescent; D000328:Adult; D000911:Antibodies, Monoclonal / Q000276:immunology; D002462:Cell Membrane / Q000737:chemistry; D002896:Chromosomes, Human, Pair 6 / Q000235:genetics; D003598:Cytoskeletal Proteins / Q000032:analysis* / Q000235:genetics / Q000276:immunology; D016189:Dystrophin / Q000032:analysis; D005091:Exons / Q000235:genetics; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008565:Membrane Proteins; D018485:Muscle Fibers, Skeletal / Q000737:chemistry* / Q000473:pathology; D009136:Muscular Dystrophies / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D010641:Phenotype; D016133:Polymerase Chain Reaction; D015854:Up-Regulation; D049411:Utrophin
D000911:Antibodies, Monoclonal; D003598:Cytoskeletal Proteins; D016189:Dystrophin; D008565:Membrane Proteins; D049411:Utrophin