PPT1 and infantile neuronal ceroid lipofuscinosis: Together, these results suggest the rapid neurodegeneration in INCL patients and in Ppt1-KO mice is likely caused, in part, by a combination of ER-stress-mediated caspase-12 activation (mediates caspase-3 activation and apoptosis (Zhang et al., 2006)) as well as elevated ROS production (causes destabilization of calcium homeostasis activating caspases-3, -9, and apoptosis) (Kim et al., 2006).