Article
The multistep hypothesis of ALS revisited: The role of genetic mutations.
Neurology - 14 Aug 2018
Chiò Adriano, Mazzini Letizia, D'Alfonso Sandra, Corrado Lucia, Canosa Antonio, Moglia Cristina, Manera Umberto, Bersano Enrica, Brunetti Maura, Barberis Marco, Veldink Jan H, van den Berg Leonard H, Pearce Neil, Sproviero William, McLaughlin Russell, Vajda Alice, Hardiman Orla, Rooney James, Mora Gabriele, Calvo Andrea, Al-Chalabi Ammar
Abstract excerpt
OBJECTIVE: Amyotrophic lateral sclerosis (ALS) incidence rates are consistent with the hypothesis that ALS is a multistep process. We tested the hypothesis that carrying a large effect mutation might account for ≥1 steps through the effect of the mutation, thus leaving fewer remaining steps before ALS begins. METHODS: We generated incidence data from an ALS population register in Italy (2007-2015) for which...
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