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

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: Guillaume Van Beersel, Eliane Tihon, Stéphane Demine, Isabelle Hamer, Michel Jadot, Thierry Arnould
Format: Article
Language:English
Published: Portland Press, Biochemical Society 2013-02-01
Series:Bioscience Reports
Subjects:
Bcl2/adenovirus E1B 19 kDa protein interacting protein 3 (BNIP3)
confocal microscopy
late infantile neuronal ceroid lipofuscinosis (LINCL)
mitochondrion
mitofusins
Online Access:http://www.bioscirep.org/bsr/033/e023/bsr033e023.htm

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http://www.bioscirep.org/bsr/033/e023/bsr033e023.htm

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