Progress towards understanding disease mechanisms in small vertebrate models of neuronal ceroid lipofuscinosis.

@article{Cooper2006ProgressTU,
  title={Progress towards understanding disease mechanisms in small vertebrate models of neuronal ceroid lipofuscinosis.},
  author={Jonathan D Cooper and Claire Russell and Hannah M Mitchison},
  journal={Biochimica et biophysica acta},
  year={2006},
  volume={1762 10},
  pages={
          873-89
        }
}
Model systems provide an invaluable tool for investigating the molecular mechanisms underlying the NCLs, devastating neurodegenerative disorders that affect the relatively inaccessible tissues of the central nervous system. These models have enabled the assessment of behavioural, pathological, cellular, and molecular abnormalities, and also allow for development and evaluation of novel therapies. This review highlights the relative advantages of the two available small vertebrate species, the… CONTINUE READING
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