Infantile neuronal ceroid lipofuscinosis (INCL) is a severe neurodegenerative disorder in
childhood that is caused by mutations in the gene encoding lysosomal palmitoyl protein
thioesterase (PPT). INCL is characterized by massive and selective loss of cortical neurons.
Here we have analyzed the intracellular processing and localization of adenovirus-mediated
PPT in mouse primary neurons and NGF-induced PC-12 cells. The neuronal processing of
PPT was found to be similar to that observed in peripheral cells, and a significant amount of …