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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...

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Detalles Bibliográficos
Autores principales: Gibertini, Sara, Ruggieri, Alessandra, Cheli, Marta, Maggi, Lorenzo
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
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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.
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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
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