Ovine neuronal ceroid lipofuscinosis: a large animal model syntenic with the human neuronal ceroid lipofuscinosis variant CLN6.

@article{Broom1998OvineNC,
  title={Ovine neuronal ceroid lipofuscinosis: a large animal model syntenic with the human neuronal ceroid lipofuscinosis variant CLN6.},
  author={Murray F. Broom and Chunjiang Zhou and Judith E. Broom and Karen J. Barwell and Robert D. Jolly and Diana F. Hill},
  journal={Journal of medical genetics},
  year={1998},
  volume={35 9},
  pages={717-21}
}
The neuronal ceroid lipofuscinoses (NCLs) are a group of inherited degenerative neurological diseases affecting children. A number of non-allelic variants have been identified within the human population and the genes for some of these have recently been identified. The underlying mechanism for the neuropathology remains an enigma; however, pioneering studies with the naturally occurring ovine model (OCL) have led to the proposal that these diseases represent lesions in specific hydrophobic… CONTINUE READING
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