Protein Quality Control Pathways at the Crossroad of Synucleinopathies

J Parkinsons Dis. 2020;10(2):369-382. doi: 10.3233/JPD-191790.

Abstract

The pathophysiology of Parkinson's disease, dementia with Lewy bodies, multiple system atrophy, and many others converge at alpha-synuclein (α-Syn) aggregation. Although it is still not entirely clear what precise biophysical processes act as triggers, cumulative evidence points towards a crucial role for protein quality control (PQC) systems in modulating α-Syn aggregation and toxicity. These encompass distinct cellular strategies that tightly balance protein production, stability, and degradation, ultimately regulating α-Syn levels. Here, we review the main aspects of α-Syn biology, focusing on the cellular PQC components that are at the heart of recognizing and disposing toxic, aggregate-prone α-Syn assemblies: molecular chaperones and the ubiquitin-proteasome system and autophagy-lysosome pathway, respectively. A deeper understanding of these basic protein homeostasis mechanisms might contribute to the development of new therapeutic strategies envisioning the prevention and/or enhanced degradation of α-Syn aggregates.

Keywords: Alpha-synuclein; autophagy; molecular chaperones; protein aggregation; protein homeostasis; synucleinopathies; ubiquitin-proteasome system.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Humans
  • Metabolic Networks and Pathways*
  • Protein Aggregation, Pathological / metabolism*
  • Synucleinopathies / metabolism*
  • alpha-Synuclein / metabolism*

Substances

  • alpha-Synuclein