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Cells 2015, 4(3), 354-386; doi:10.3390/cells4030354

Autophagy and Neurodegeneration: Insights from a Cultured Cell Model of ALS

Institute of Neuroscience, Consiglio Nazionale delle Ricerche, and Department of Medical Biotechnology and Translational Medicine (BIOMETRA), Università degli Studi di Milano, Via Vanvitelli 32, 20129 Milano, Italy
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Author to whom correspondence should be addressed.
Academic Editor: Anne Hamacher-Brady
Received: 20 May 2015 / Revised: 7 July 2015 / Accepted: 27 July 2015 / Published: 6 August 2015
(This article belongs to the Special Issue Autophagy)
View Full-Text   |   Download PDF [3360 KB, uploaded 7 August 2015]   |  

Abstract

Autophagy plays a major role in the elimination of cellular waste components, the renewal of intracellular proteins and the prevention of the build-up of redundant or defective material. It is fundamental for the maintenance of homeostasis and especially important in post-mitotic neuronal cells, which, without competent autophagy, accumulate protein aggregates and degenerate. Many neurodegenerative diseases are associated with defective autophagy; however, whether altered protein turnover or accumulation of misfolded, aggregate-prone proteins is the primary insult in neurodegeneration has long been a matter of debate. Amyotrophic lateral sclerosis (ALS) is a fatal disease characterized by selective degeneration of motor neurons. Most of the ALS cases occur in sporadic forms (SALS), while 10%–15% of the cases have a positive familial history (FALS). The accumulation in the cell of misfolded/abnormal proteins is a hallmark of both SALS and FALS, and altered protein degradation due to autophagy dysregulation has been proposed to contribute to ALS pathogenesis. In this review, we focus on the main molecular features of autophagy to provide a framework for discussion of our recent findings about the role in disease pathogenesis of the ALS-linked form of the VAPB gene product, a mutant protein that drives the generation of unusual cytoplasmic inclusions. View Full-Text
Keywords: autophagy; UPS; protein degradation; autophagy receptors; proteostasis; cytoplasmic inclusions; aggregates; neurodegeneration; ALS; VAPB autophagy; UPS; protein degradation; autophagy receptors; proteostasis; cytoplasmic inclusions; aggregates; neurodegeneration; ALS; VAPB
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).

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MDPI and ACS Style

Navone, F.; Genevini, P.; Borgese, N. Autophagy and Neurodegeneration: Insights from a Cultured Cell Model of ALS. Cells 2015, 4, 354-386.

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