Successive neuron loss in the thalamus and cortex in a mouse model of infantile neuronal ceroid lipofuscinosis.

@article{Kielar2007SuccessiveNL,
  title={Successive neuron loss in the thalamus and cortex in a mouse model of infantile neuronal ceroid lipofuscinosis.},
  author={Catherine Kielar and Lucy Maddox and Ellen Bible and Charlie C Pontikis and Shannon L Macauley and Megan A Griffey and Michael Wong and Mark S Sands and Jonathan D Cooper},
  journal={Neurobiology of disease},
  year={2007},
  volume={25 1},
  pages={150-62}
}
Infantile neuronal ceroid lipofuscinosis (INCL) is caused by deficiency of the lysosomal enzyme, palmitoyl protein thioesterase 1 (PPT1). We have investigated the onset and progression of pathological changes in Ppt1 deficient mice (Ppt1-/-) and the development of their seizure phenotype. Surprisingly, cortical atrophy and neuron loss occurred only late in… CONTINUE READING