Mouse Models of Spinocerebellar Ataxia Type 3 (Machado-Joseph Disease)

@article{Gould2012MouseMO,
  title={Mouse Models of Spinocerebellar Ataxia Type 3 (Machado-Joseph Disease)},
  author={Veronica F. Colomer Gould},
  journal={Neurotherapeutics},
  year={2012},
  volume={9},
  pages={285-296}
}
Machado-Joseph disease, also called spinocerebellar ataxia type 3 (MJD/SCA3), is a hereditary and neurodegenerative movement disorder caused by ataxin-3 with a pathological polyglutamine stretch (mutant ataxin-3). Seven transgenic mouse models expressing full-length human mutant ataxin-3 throughout the brain have been generated and are compared in this review. They vary in the corresponding transgenic DNA constructs with differences that include the encoded human ataxin-3 isoform(s), number of… CONTINUE READING
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