Protein Aggregates and Aggrephagy in Myopathies

Int J Mol Sci. 2023 May 8;24(9):8456. doi: 10.3390/ijms24098456.

Abstract

A number of muscular disorders are hallmarked by the aggregation of misfolded proteins within muscle fibers. A specialized form of macroautophagy, termed aggrephagy, is designated to remove and degrade protein aggregates. This review aims to summarize what has been studied so far about the direct involvement of aggrephagy and the activation of the key players, among others, p62, NBR1, Alfy, Tollip, Optineurin, TAX1BP1 and CCT2 in muscular diseases. In the first part of the review, we describe the aggrephagy pathway with the involved proteins; then, we illustrate the muscular disorder histologically characterized by protein aggregates, highlighting the role of aggrephagy pathway abnormalities in these muscular disorders.

Keywords: aggrephagy; aggresome; muscle disorders; protein aggregates; protein quality control (PQC).

Publication types

  • Review

MeSH terms

  • Apoptosis Regulatory Proteins
  • Autophagy
  • Humans
  • Macroautophagy*
  • Muscular Diseases*
  • Protein Aggregates

Substances

  • Protein Aggregates
  • Apoptosis Regulatory Proteins

Grants and funding

This research received no external funding.