Abstract:Background
Poly-GA, a dipeptide repeat protein unconventionally translated from GGGGCC repeat expansions in C9orf72, is abundant in C9orf72-related amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Although the poly-GA aggregates have been identified in C9orf72-ALS/FTD neurons, the effects on UPS and autophagy, and their exact molecular mechanisms have not been fully elucidated.
Results
Herein, our in vivo experiments show that ploy-GA mice expressing 150 repeats but not 30 repeats GA ex… Show more
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