Article
Cell-Clearing Systems Bridging Repeat Expansion Proteotoxicity and Neuromuscular Junction Alterations in ALS and SBMA.
International journal of molecular sciences - 4 Jun 2020
Limanaqi Fiona, Busceti Carla Letizia, Biagioni Francesca, Cantini Federica, Lenzi Paola, Fornai Francesco
Abstract excerpt
The coordinated activities of autophagy and the ubiquitin proteasome system (UPS) are key to preventing the aggregation and toxicity of misfold-prone proteins which manifest in a number of neurodegenerative disorders. These include proteins which are encoded by genes containing nucleotide repeat expansions. In the present review we focus on the overlapping role of autophagy and the UPS in repeat expansion...
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