We subsequently tested two distinct murine models of NPC1 deficiency for their phenotypic response to HDAC inhibitors: The Npc1nih variant, defined by a null allele of Npc1, and Npc1nmf164 (a missense allele of NPC1 comprising a D1005G protein variation); both animal lines develop a chronic, debilitating neurological disease and die between 9-12 and 13-16 weeks of age, respectively (the missense allele confers a milder, later onset, form of the disease). Here, NPC1 is linked to nervous system disorder.