Different molecular mechanisms involved in spontaneous and oxidative stress-induced mitochondrial fragmentation in tripeptidyl peptidase-1 (TPP-1)-deficient fibroblasts

NCLs (neuronal ceroid lipofuscinoses) form a group of eight inherited autosomal recessive diseases characterized by the intralysosomal accumulation of autofluorescent pigments, called ceroids. Recent data suggest that the pathogenesis of NCL is associated with the appearance of fragmented mitochondr...

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Bibliographic Details
Main Authors: Van Beersel, Guillaume, Tihon, Eliane, Demine, Stéphane, Hamer, Isabelle, Jadot, Michel, Arnould, Thierry
Format: Online
Language:English
Published: Portland Press Ltd. 2013
Online Access:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3566540/