Use of model organisms for the study of neuronal ceroid lipofuscinosis.

  title={Use of model organisms for the study of neuronal ceroid lipofuscinosis.},
  author={Michael E. Bond and Sophia-Martha kleine Holthaus and Imke Tammen and Guy Tear and Claire Russell},
  journal={Biochimica et biophysica acta},
  volume={1832 11},
Neuronal ceroid lipofuscinoses are a group of fatal progressive neurodegenerative diseases predominantly affecting children. Identification of mutations that cause neuronal ceroid lipofuscinosis, and subsequent functional and pathological studies of the affected genes, underpins efforts to investigate disease mechanisms and identify and test potential therapeutic strategies. These functional studies and pre-clinical trials necessitate the use of model organisms in addition to cell and tissue… CONTINUE READING

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