PPT1 and infantile neuronal ceroid lipofuscinosis: Given the previous reports of impaired adaptive energy metabolism and mitochondrial dysfunction in INCL and other neurodegenerative diseases [13], [14], we hypothesized that the Ppt1-KO mice would not upregulate PGC-1α and uncoupling protein 1 (UCP1) in brown adipose tissue and therefore would have impaired thermogenesis and thermoregulation.