Developmental changes in the expression of neuronal ceroid lipofuscinoses-linked proteins.

@article{Suopanki2000DevelopmentalCI,
  title={Developmental changes in the expression of neuronal ceroid lipofuscinoses-linked proteins.},
  author={Jaana Suopanki and Sanna Partanen and Junji Ezaki and Marc Baumann and Eiki Kominami and Jaana Tyynel{\"a}},
  journal={Molecular genetics and metabolism},
  year={2000},
  volume={71 1-2},
  pages={190-4}
}
Neuronal ceroid lipofuscinoses (NCL) form a distinct group of storage diseases where the normal development of the central nervous system is interrupted and neurons of the neocortex begin to degenerate. Mutations in genes encoding three lysosomal enzymes are the causes for three early-onset forms of NCLs: palmitoyl-protein thioesterase 1 (PPT1) is deficient in human infantile NCL, tripeptidyl peptidase 1 (TTP1) in late-infantile NCL, and cathepsin D in congenital ovine NCL. We wanted to compare… CONTINUE READING