“…For instance, in patient derived NPC fibroblasts, ROS generation has been shown to be decreased [33], while for NCL human fibroblasts --associated with CLN2 and CLN3 [58] -and GD human fibroblasts [31], production of superoxide and hydrogen peroxide were found to be increased. In different studies using NCL human lymphoblasts [46], a yeast NPC model [32] or NPC human fibroblasts [69], a human oligodendryocyte cell line exposed to psychosine to model Krabbe disease [73], or mytoubes derived from a knockout mice model for Pompe disease [51], basal ROS and/or H 2 O 2 production were also increased. Of note, increased protein oxidation and lipid peroxidation, as well as DNA damage due to oxidative stress, were reported in the urine, plasma and leukocytes of MPS IV patients [68], in a yeast NPC model, in NPC human fibroblasts and in different NPC mice tissues [32,69,70], as well as in brain tissues of GM2 gangliosidosis mice [30] and in NCL patients [71].…”