Our genetic studies, with validation in mammalian cell culture system including C9-ALS/FTD patient fibroblasts, uncovered the mTORC2/AKT signaling axis regulating the RQC of C9-ALS/FTD-associated poly(GR) translation as a common cellular pathway involved in ALS/FTD. The gene discussed is AKT1; the disease is amyotrophic lateral sclerosis.