Aiming for the treatment of INCL, previous studies reported that applying NtBuHA (23) or N-acetylcysteine (12), which could unspecifically break the thioester-linkage connecting palmitate and modified protein in order to ease the burden of hyperpalmitoylation due to PPT1 dysfunction in PPT1-KO mice (12, 32), was proven to be effective. Here, PPT1 is linked to infantile neuronal ceroid lipofuscinosis.