Abstract
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.
MeSH terms
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Adolescent
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Adult
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Antibodies, Monoclonal / immunology
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Cell Membrane / chemistry
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Chromosomes, Human, Pair 6 / genetics
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Cytoskeletal Proteins / analysis*
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Cytoskeletal Proteins / genetics
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Cytoskeletal Proteins / immunology
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Dystrophin / analysis
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Exons / genetics
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Humans
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Immunohistochemistry
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Male
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Membrane Proteins*
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Muscle Fibers, Skeletal / chemistry*
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Muscle Fibers, Skeletal / pathology
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Muscular Dystrophies / genetics
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Muscular Dystrophies / metabolism*
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Muscular Dystrophies / pathology
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Phenotype
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Polymerase Chain Reaction
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Up-Regulation
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Utrophin
Substances
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Antibodies, Monoclonal
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Cytoskeletal Proteins
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Dystrophin
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Membrane Proteins
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Utrophin