Genome-encoded Cytoplasmic Double-Stranded RNAs, Found in C9ORF72 ALS-FTD Brain, Provoke Propagated Neuronal Death
Steven Rodriguez, Benjamin R. Schrank, Aslı Şahin, Hawra J. Al Lawati, Isabel M. Costantino, Eric G. Benz, Darian Fard, Alefiya Dhilla Albers, Luxiang Cao, Alexis C. Gomez, Elena Ratti, Merit Cudkowicz, Matthew P. Frosch, Michael E. Talkowski, Peter Karl Sorger, , Mark W. Albers
The paper shows that cytoplasmic double-stranded RNA (cdsRNA) from C9ORF72 expansions in ALS-FTD brains triggers IFN-I signaling and neuronal death through a viral-mimetic mechanism.
bioRxiv · Jan 19, 2018 · PreprintNeuroscience