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

@article{Ct1999ChimaericMD,
  title={Chimaeric mice deficient in dystroglycans develop muscular dystrophy and have disrupted myoneural synapses},
  author={Patrice D. C{\^o}t{\'e} and Hakima Moukhles and Michael Lindenbaum and Salvatore Carbonetto},
  journal={Nature Genetics},
  year={1999},
  volume={23},
  pages={338-342}
}
Mutations in the dystrophin gene (DMD) and in genes encoding several dystrophin-associated proteins result in Duchenne and other forms of muscular dystrophy. α-Dystroglycan (Dg) binds to laminins in the basement membrane surrounding each myofibre and docks with β-Dg, a transmembrane protein, which in turn interacts with dystrophin or utrophin in the subplasmalemmal cytoskeleton. α- and β-Dgs are thought to form the functional core of a larger complex of proteins extending from the basement… CONTINUE READING
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