MRI Reveals Differences in Neuroanatomy of Mouse Models of NPC Disease

John Totenhagen¹, Eriko Yoshimaru¹, Ivan Borbon², Christy Howison³, Robert Erickson², Theodore Trouard¹

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.