A zebrafish model of CLN2 disease is deficient in tripeptidyl peptidase 1 and displays progressive neurodegeneration accompanied by a reduction in proliferation.

@article{Mahmood2013AZM,
  title={A zebrafish model of CLN2 disease is deficient in tripeptidyl peptidase 1 and displays progressive neurodegeneration accompanied by a reduction in proliferation.},
  author={Fahad Mahmood and Sonia Fu and Jennifer Cooke and Stephen W. Wilson and Jonathan D Cooper and Claire Russell},
  journal={Brain : a journal of neurology},
  year={2013},
  volume={136 Pt 5},
  pages={1488-507}
}
Tripeptidyl peptidase 1 (TPP1) deficiency causes CLN2 disease, late infantile (or classic late infantile neuronal ceroid lipofuscinosis), a paediatric neurodegenerative disease of autosomal recessive inheritance. Patients suffer from blindness, ataxia, epilepsy and cognitive defects, with MRI indicating widespread brain atrophy, and profound neuron loss is evident within the retina and brain. Currently there are no effective therapies for this disease, which causes premature death in… CONTINUE READING