John Totenhagen1, Eriko Yoshimaru1,
Ivan Borbon2, Christy Howison3, Robert Erickson2,
Theodore Trouard1
1Biomedical Engineering,
University of Arizona, Tucson, AZ, United States; 2Pediatrics,
University of Arizona, Tucson, AZ, United States; 3Arizona
Research Laboratories, University of Arizona, Tucson, AZ, United States
Niemann Pick Type C (NPC) disease is a rare genetic neurodegenerative disease with no effective treatment or cure. The commonly used mouse model of NPC disease (Npc1-/-) has been employed in many treatment studies and shows drastic dysmyelination and increased ventricular volume compared to control mice. A new mouse model of NPC disease has recently been made available which more closely mimics the genetic defect found in human NPC disease. MRI measurements of this new mouse model are presented here for the first time and show marked differences, particularly in white matter regions, compared to the Npc1-/- mouse.