Alteration of hepatic gene expression profile in NPC mice correlates with tissue damage and oxidative stress status

ABSTRACT: Background: Niemann Pick type C disease (NPC) is a neurovisceral lipid storage disorder, characterized by unesterified cholesterol accumulation in lysosomal/late endosome compartments. The main tissues affected in the disease are the liver and the cerebellum. Although the primary defect in this disease occurs in the liver, the pathological mechanisms involved in hepatocyte damage and death have been poorly explored. Here, we assessed biochemical parameters, liver histology and the mRNA abundance of genes encoding proteins associated to oxidative stress, copper metabolism, fibrosis and inflammation and cholesterol metabolism in livers of WT and NPC mice. Methodology/Principal Findings: We found that the histology of NPC mice livers show signals of inflammation and fibrosis. This was corr

ORGANISM(S): Mus musculus

SUBMITTER: Leonardo Pavez 

PROVIDER: E-GEOD-24013 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress