NPC1 and Ataxia: Metabolites were measured by 1H-NMR spectroscopy in the cerebella, cerebral cortices and hippocampi of BALB/cNctr-Npc1m1N/J mice, a well-characterized murine model of NPC disease with a null mutation in Npc1. Homozygous Npc1-/- mice are asymptomatic at birth, but develop progressive ataxia, tremors and hindlimb dystonia around 5 – 6 weeks of age, and have an average lifespan of approximately 12 weeks of age [33].