Ontology highlight
ABSTRACT:
SUBMITTER: Ferraiuolo L
PROVIDER: S-EPMC5081600 | biostudies-literature | 2016 Oct
REPOSITORIES: biostudies-literature
Ferraiuolo Laura L Meyer Kathrin K Sherwood Thomas W TW Vick Jonathan J Likhite Shibi S Frakes Ashley A Miranda Carlos J CJ Braun Lyndsey L Heath Paul R PR Pineda Ricardo R Beattie Christine E CE Shaw Pamela J PJ Askwith Candice C CC McTigue Dana D Kaspar Brian K BK
Proceedings of the National Academy of Sciences of the United States of America 20160929 42
Oligodendrocytes have recently been implicated in the pathophysiology of amyotrophic lateral sclerosis (ALS). Here we show that, in vitro, mutant superoxide dismutase 1 (SOD1) mouse oligodendrocytes induce WT motor neuron (MN) hyperexcitability and death. Moreover, we efficiently derived human oligodendrocytes from a large number of controls and patients with sporadic and familial ALS, using two different reprogramming methods. All ALS oligodendrocyte lines induced MN death through conditioned m ...[more]