Oxidative stress (OS) represents a state of an imbalanced amount of reactive oxygen
species (ROS) and/or a hampered efficacy of the antioxidative defense system. Cells of the …

Glutathione (GSH) is an antioxidant thiol that has a vital role in the pathogenesis of various
human diseases such as cardiovascular disease and cancer. Hence, it is necessary to study …

Niemann–Pick type C disease (NPCD) is a rare neurodegenerative disorder most commonly
caused by mutations in the lysosomal protein Niemann–Pick C1 (NPC1), which is implicated …

Niemann-Pick type C disease (NPCD) is a lysosomal storage disorder caused by mutations
in the NPC1 gene. The most affected tissues are the central nervous system and liver, and …

Liver cancer is a disease with high mortality; it is often diagnosed at intermediate and
advanced stages and has a high recurrence rate. ROS restriction and adequate energy …

Abstract Analysis of endogenous metabolites in various diseases is useful for searching
diagnostic biomarkers and elucidating the molecular mechanisms of pathophysiology. The …

Purpose: To characterize two mitochondrial membrane transporters 2-oxoglutarate (OGC)
and dicarboxylate (DIC) in human RPE (hRPE) and to elucidate their role in the regulation of …

Objectives: Hypercholesterolaemia has been implicated in the pathogenesis of
neurodegenerative diseases. In this work, we tested whether cholesterol-mediated …

Abnormal cholesterol metabolism is linked to many neurodegenerative disorders. Here, we
present a protocol for the production of a recombinant protein consisting of a Glutathione-S …

Abstract Niemann-Pick type C (NPC) is an autosomal recessive lysosomal storage disorder
due to mutations in NPC1 (95% cases) or NPC2 genes, encoding NPC1 and NPC2 proteins …