Quality-control mechanisms targeting translationally stalled and C-terminally extended poly(GR) associated with ALS/FTD

Abstract excerpt

Significance Amyotrophic laterosclerosis (ALS) is a rapidly progressing neurological disease that robs patients’ motor functions. Despite intensive research, molecular events that initiate the disease are poorly understood. Expansion of G4C2 repeats in the C9orf72 gene causes ALS with frontotemporal dementia, one of the most common forms of ALS. Increasing evidence suggests that dipeptides translated from G4C2...

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