Relative efficacy of nicotinamide treatment of a mouse model of infantile Niemann-Pick C1 disease

• Main Authors: Marshall, Craig A., Borbon, Ivan A., Erickson, Robert P.
• Other Authors: Department of Pediatrics, University of Arizona
• Language: en
• Published: SPRINGER HEIDELBERG 2016
• Subjects: Histone deacetylase; Nicotinamide; Niemann-Pick C1 disease; Oxidative stress; Sirtuin; Valproic acid
• Online Access: http://hdl.handle.net/10150/622828; http://arizona.openrepository.com/arizona/handle/10150/622828

Nicotinamide delivered in drinking water at about 2 g/kg/day significantly prolonged survival and showed a suggestive improvement on memory in the Npc1 (nih) / Npc1 (nih) mouse model of infantile NPC1 disease. It is likely that this role is due to its function as a histone deacetylase (HDAC) inhibitor although another HDAC inhibitor, valproic acid, was without effect. Nicotinamide could also work by preventing/reversing oxidative stress.