Article
Excitatory neuron-specific suppression of the integrated stress response contributes to autism-related phenotypes in fragile X syndrome.
Neuron - 4 Oct 2023
Hooshmandi Mehdi, Sharma Vijendra, Thörn Perez Carolina, Sood Rapita, Krimbacher Konstanze, Wong Calvin, Lister Kevin C, Ureña Guzmán Alba, Bartley Trevor D, Rocha Cecilia, Maussion Gilles, Nadler Emma, Roque Patricia Margarita, Gantois Ilse, Popic Jelena, Lévesque Maxime, Kaufman Randal J, Avoli Massimo, Sanz Elisenda, Nader Karim, Hagerman Randi Jenssen, Durcan Thomas M, Costa-Mattioli Mauro, Prager-Khoutorsky Masha, Lacaille Jean-Claude, Martinez-Cerdeno Veronica, Gibson Jay R, Huber Kimberly M, Sonenberg Nahum, Gkogkas Christos G, Khoutorsky Arkady
Abstract excerpt
Dysregulation of protein synthesis is one of the key mechanisms underlying autism spectrum disorder (ASD). However, the role of a major pathway controlling protein synthesis, the integrated stress response (ISR), in ASD remains poorly understood. Here, we demonstrate that the main arm of the ISR, eIF2α phosphorylation (p-eIF2α), is suppressed in excitatory, but not inhibitory, neurons in a mouse model of fragile...
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