Liver magnetic resonance spectroscopy as an alternative for evaluating Niemann-Pick C disease progression.

ABSTRACT: Niemann-Pick disease (NP) is a group of rare genetic disorders that affect normal lipid metabolism and cause an accumulation of lipids in the liver, spleen, brain, and bone marrow. NP patients develop brain alterations and a very fast progression of liver damage. The purpose of this study is to characterize the changes in liver lipid composition during the progression of this disease using ex vivo magnetic resonance spectroscopy (MRS) in mouse models with the aim of identifying potential biomarkers to support a future non-invasive technique to follow-up these patients. NP type C (NPC) and wild-type (WT) mice were fed a chow diet and euthanized at 5 weeks of age (n = 5 per group) and 9 weeks of age (n = 5 per group). We extracted lipids from their livers and analyzed them with

SUBMITTER: Xavier A 

PROVIDER: S-EPMC11800100 | biostudies-literature | 2025 Feb

REPOSITORIES: biostudies-literature