… 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 …

… 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 …

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

… chronically has been shown to be able to replenish mGSH levels due to its effect on the …
demonstrating the potential therapeutic effects of oral administration of GSH in practical dose for …

… LSD, Niemann–Pick disease, type C (NPC), … mitochondrial GSH would be expected to
leave the enzymes of MRC, the phospholipid milieu of the inner mitochondrial and mitochondrial …

… , and depletion of glutathione (Figure 2C), confirming … mitochondrial dysfunction in NPC
liver cells. These proteins might be new potential targets for the treatment of NPC hepatic disease…

… Here we focus on one essential aspect: the role of mitochondria in … In mitochondria the
GSH is mainly in its reduced form and represents a minor portion of the total cellular glutathione (…

… dysfunctional mitochondria could be a potential therapeutic … example Gaucher Disease
(GD) and Niemann Pick Type C (… stress, in addition to replenishing cerebral ATP levels and …