Article
TDP-43 loss-of-function causes neuronal loss due to defective steroid receptor-mediated gene program switching in Drosophila.
Cell reports - 31 Jan 2013
Vanden Broeck Lies, Naval-Sánchez Marina, Adachi Yoshitsugu, Diaper Danielle, Dourlen Pierre, Chapuis Julien, Kleinberger Gernot, Gistelinck Marc, Van Broeckhoven Christine, Lambert Jean-Charles, Hirth Frank, Aerts Stein, Callaerts Patrick, Dermaut Bart
Abstract excerpt
TDP-43 proteinopathy is strongly implicated in the pathogenesis of amyotrophic lateral sclerosis and related neurodegenerative disorders. Whether TDP-43 neurotoxicity is caused by a novel toxic gain-of-function mechanism of the aggregates or by a loss of its normal function is unknown. We increased and decreased expression of TDP-43 (dTDP-43) in Drosophila. Although upregulation of dTDP-43 induced neuronal...
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