Chimaeric mice deficient in dystroglycans develop muscular dystrophy and have disrupted myoneural synapses

Mutations in the dystrophin gene 1 ( DMD ) and in genes encoding several dystrophin-associated proteins result in Duchenne and other forms of muscular dystrophy 2 . α-Dystroglycan (Dg) binds to laminins in the basement membrane surrounding each myofibre and docks with β-Dg, a transmembrane protein,...

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Veröffentlicht in:	Nature genetics 1999-11, Vol.23 (3), p.338-342
Hauptverfasser:	Moukhles, Hakima, Carbonetto, Salvatore, Côté, Patrice D, Lindenbaum, Michael
Format:	Artikel
Sprache:	eng
Schlagworte:	a-dystroglycan Agriculture Animal Genetics and Genomics Animals Basement Membrane - metabolism Basement Membrane - ultrastructure Biological and medical sciences Biomedical and Life Sciences Biomedicine Cancer Research Chimera - genetics Chimera - physiology Cytoskeletal Proteins - deficiency Cytoskeletal Proteins - genetics Cytoskeletal Proteins - metabolism Cytoskeleton - metabolism Cytoskeleton - ultrastructure Diseases of striated muscles. Neuromuscular diseases DMD gene Dystroglycans dystrophin Dystrophin - metabolism Gene Function Hindlimb - innervation Hindlimb - metabolism Hindlimb - pathology Human Genetics Humans Laminin - metabolism letter Medical sciences Membrane Glycoproteins - deficiency Membrane Glycoproteins - genetics Membrane Glycoproteins - metabolism Membrane Proteins - metabolism Mice Mice, Transgenic Microscopy, Electron Muscle, Skeletal - innervation Muscle, Skeletal - metabolism Muscle, Skeletal - pathology Muscle, Skeletal - ultrastructure Muscular Dystrophies - genetics Muscular Dystrophies - metabolism Muscular Dystrophies - pathology Mutation - genetics Neurology Neuromuscular Junction - metabolism Neuromuscular Junction - pathology Neuromuscular Junction - ultrastructure Receptor Aggregation Receptors, Cholinergic - metabolism Sarcoglycans Stem Cells - metabolism Utrophin
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