This notion is supported by findings that fibroblasts from INCL patients and brain tissue from mice lacking PPT1 have significantly decreased levels of the peroxisome proliferator-activated receptor-γ coactivator-1α (PGC-1α) and of NAD-dependent deacetylase sirtuin 1 [13]. Here, PPT1 is linked to infantile neuronal ceroid lipofuscinosis.