Cargando…
Protein Aggregates and Aggrephagy in Myopathies
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 in...
Autores principales: | , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2023
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10179229/ https://www.ncbi.nlm.nih.gov/pubmed/37176163 http://dx.doi.org/10.3390/ijms24098456 |
_version_ | 1785041048533204992 |
---|---|
author | Gibertini, Sara Ruggieri, Alessandra Cheli, Marta Maggi, Lorenzo |
author_facet | Gibertini, Sara Ruggieri, Alessandra Cheli, Marta Maggi, Lorenzo |
author_sort | Gibertini, Sara |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-10179229 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-101792292023-05-13 Protein Aggregates and Aggrephagy in Myopathies Gibertini, Sara Ruggieri, Alessandra Cheli, Marta Maggi, Lorenzo Int J Mol Sci Review 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. MDPI 2023-05-08 /pmc/articles/PMC10179229/ /pubmed/37176163 http://dx.doi.org/10.3390/ijms24098456 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Review Gibertini, Sara Ruggieri, Alessandra Cheli, Marta Maggi, Lorenzo Protein Aggregates and Aggrephagy in Myopathies |
title | Protein Aggregates and Aggrephagy in Myopathies |
title_full | Protein Aggregates and Aggrephagy in Myopathies |
title_fullStr | Protein Aggregates and Aggrephagy in Myopathies |
title_full_unstemmed | Protein Aggregates and Aggrephagy in Myopathies |
title_short | Protein Aggregates and Aggrephagy in Myopathies |
title_sort | protein aggregates and aggrephagy in myopathies |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10179229/ https://www.ncbi.nlm.nih.gov/pubmed/37176163 http://dx.doi.org/10.3390/ijms24098456 |
work_keys_str_mv | AT gibertinisara proteinaggregatesandaggrephagyinmyopathies AT ruggierialessandra proteinaggregatesandaggrephagyinmyopathies AT chelimarta proteinaggregatesandaggrephagyinmyopathies AT maggilorenzo proteinaggregatesandaggrephagyinmyopathies |