Abstract
Duchenne muscular dystrophy is a severe X chromosome-linked, muscle-wasting disease caused by lack of the protein dystrophin. The exact function of dystrophin remains to be determined. However, analysis of its interaction with a large oligomeric protein complex at the sarcolemma and the identification of a structurally related protein, utrophin, is leading to the characterization of candidate genes for other neuromuscular disorders.
Publication types
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Research Support, Non-U.S. Gov't
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Review
MeSH terms
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Animals
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Calcium-Binding Proteins
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Chromosome Mapping
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Cytoskeletal Proteins / physiology*
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Dystroglycans
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Dystrophin / physiology*
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Genes
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Humans
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Macromolecular Substances
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Membrane Glycoproteins / physiology*
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Membrane Proteins / physiology*
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Muscle Proteins / physiology*
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Muscles / physiology*
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Neuromuscular Junction / physiology
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Utrophin
Substances
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Calcium-Binding Proteins
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Cytoskeletal Proteins
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DAG1 protein, human
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Dystrophin
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Macromolecular Substances
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Membrane Glycoproteins
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Membrane Proteins
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Muscle Proteins
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Utrophin
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syntrophin alpha1
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Dystroglycans