Ontology highlight
ABSTRACT:
SUBMITTER: Wang W
PROVIDER: S-EPMC3820133 | biostudies-literature | 2013 Dec
REPOSITORIES: biostudies-literature
Wang Wenzhang W Li Li L Lin Wen-Lang WL Dickson Dennis W DW Petrucelli Leonard L Zhang Teng T Wang Xinglong X
Human molecular genetics 20130704 23
Mutations in TDP-43 lead to familial ALS. Expanding evidence suggests that impaired mitochondrial dynamics likely contribute to the selective degeneration of motor neurons in SOD1-associated ALS. In this study, we investigated whether and how TDP-43 mutations might impact mitochondrial dynamics and function. We demonstrated that overexpression of wild-type TDP-43 resulted in reduced mitochondrial length and density in neurites of primary motor neurons, features further exacerbated by ALS-associa ...[more]