Excessive proteostasis contributes to pathology in fragile X syndrome

Susana R Louros 1 , Sang S Seo 1 , Beatriz Maio 1 , Cristina Martinez-Gonzalez 1 , Miguel A Gonzalez-Lozano 2 , Melania Muscas 1 , Nick C Verity 1 , Jimi C Wills 3 , Ka Wan Li 2 , Matthew F Nolan 1 , Emily K Osterweil 4

Over-synthesis of proteins in neurons is a core problem in fragile X syndrome, the leading identified monogenic cause of autism. Here, the authors now show overdegradation
of proteins is also a contributor to neuropathology. This indicates that the proteostasis cycle is elevated and that reducing protein degradation is a potential new therapeutic strategy.