… into mitochondria, resulting in mitochondrial GSH (mGSH) depletion, we investigated the
impact of mGSH recovery in NPC disease. We show that GSH ethyl ester (GSH-EE), but not N-…

… mitochondrial function, which can contribute to the progression not only of AD but also of the
lysosomal storage disorder Niemann–Pick type C (NPC) disease, … replenishment with GSH …

… membrane fluidity and subsequent replenishment of mitochondrial GSH levels. In vivo …
potential therapeutic approach for the treatment of NPC disease by increasing brain mitochondrial …

… ROS and a decrease in the endogenous antioxidant glutathione (GSH), … focus of the present
review, in primary OLs modeling the maturational arrest characteristic of NPC, mitochondria …

… homeostasis, while Niemann-Pick type C (NPC) disease is caused … of GSH into mitochondria,
resulting in mitochondrial GSH … potential beneficial effects of mGSH replenishment by GSH …

… upon mitochondrial cholesterol extraction by HPβCD (Yu et al., 2005). Replenishment of
mitochondrial GSH with GSH … reticulum transport of cholesterol in Niemann-Pick type C cells …

… in increased mitochondrial GSH levels, improved mitochondrial … , replenishment of mGSH
levels improved mitochondrial … to mitochondria may be of relevance in NPC disease. The …

… (SM) homeostasis, while Niemann-Pick type C (NPC) disease is caused by … Mitochondrial
GSH replenishment as a potential therapeutic approach for Niemann Pick type C disease…

… and molecular disease mechanisms and to test potential therapeutic approaches at the …
unesterified cholesterol, and showed impaired mitochondrial function and defective autophagy. …