Protective mechanism in NPC1 – the role of oxLDL

Abstract

The Niemann Pick disease type C1 (NPC1) is a lysosomal storage disorder caused by a mutation in the NPC1 gene. The mutation disrupts the normal cholesterol traficking and induces cholesterol accumulation in cells of the liver and the brain. Till now, no therapy exists for NPC1 patients. But recently, pneumococci immunization has shown to reduce inflammation in Non-alcoholic steatohepatitis (NASH), another lipid storage disorder. Therefore, pneumococci immunization was also tested on NPC1mut mice. The results indicate an improved pathology in the NPC1 mice after the immunization