Article
Mutant TDP-43 does not impair mitochondrial bioenergetics in vitro and in vivo.
Molecular neurodegeneration - 8 May 2017
Kawamata Hibiki, Peixoto Pablo, Konrad Csaba, Palomo Gloria, Bredvik Kirsten, Gerges Meri, Valsecchi Federica, Petrucelli Leonard, Ravits John M, Starkov Anatoly, Manfredi Giovanni
Abstract excerpt
BACKGROUND: Mitochondrial dysfunction has been linked to the pathogenesis of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). Functional studies of mitochondrial bioenergetics have focused mostly on superoxide dismutase 1 (SOD1) mutants, and showed that mutant human SOD1 impairs mitochondrial oxidative phosphorylation, calcium homeostasis, and dynamics. However, recent reports...
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