Annals of Clinical and Translational

doi: 10.1002/acn3.261 Objective: Oxidative stress in the brain is highly prevalent in many neurode-generative disorders including lysosomal storage disorders, in which neurode-generation is a devastating manifestation. Despite intense studies, a precise mechanism linking oxidative stress to neuropathology in specific neurodegener-ative diseases remains largely unclear. Methods: Infantile neuronal ceroid lipo-fuscinosis (INCL) is a devastating neurodegenerative lysosomal storage disease caused by mutations in the ceroid lipofuscinosis neuronal-1 (CLN1) gene encoding palmitoyl-protein thioesterase-1. Previously, we reported that in the brain of Cln1/ mice, which mimic INCL, and in postmortem brain tissues from INCL patients, increased oxidative stress is readily detectable. We used molecular, biochemical, immunohistological, and electrophysiological analyses of brain tissues of Cln1/ mice to study the role(s) of oxidative stress in medi-ating neuropathology. Results: Our results show that in Cln1/ mice oxidativ