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Valosin Containing Protein (VCP): A Multistep Regulator of Autophagy

Valosin containing protein (VCP) has emerged as a central protein in the regulation of the protein quality control (PQC) system. VCP mutations are causative of multisystem proteinopathies, which include neurodegenerative diseases (NDs), and share various signs of altered proteostasis, mainly associa...

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Autores principales: Ferrari, Veronica, Cristofani, Riccardo, Tedesco, Barbara, Crippa, Valeria, Chierichetti, Marta, Casarotto, Elena, Cozzi, Marta, Mina, Francesco, Piccolella, Margherita, Galbiati, Mariarita, Rusmini, Paola, Poletti, Angelo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
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Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8878954/
https://www.ncbi.nlm.nih.gov/pubmed/35216053
http://dx.doi.org/10.3390/ijms23041939
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author Ferrari, Veronica
Cristofani, Riccardo
Tedesco, Barbara
Crippa, Valeria
Chierichetti, Marta
Casarotto, Elena
Cozzi, Marta
Mina, Francesco
Piccolella, Margherita
Galbiati, Mariarita
Rusmini, Paola
Poletti, Angelo
author_facet Ferrari, Veronica
Cristofani, Riccardo
Tedesco, Barbara
Crippa, Valeria
Chierichetti, Marta
Casarotto, Elena
Cozzi, Marta
Mina, Francesco
Piccolella, Margherita
Galbiati, Mariarita
Rusmini, Paola
Poletti, Angelo
author_sort Ferrari, Veronica
collection PubMed
description Valosin containing protein (VCP) has emerged as a central protein in the regulation of the protein quality control (PQC) system. VCP mutations are causative of multisystem proteinopathies, which include neurodegenerative diseases (NDs), and share various signs of altered proteostasis, mainly associated with autophagy malfunctioning. Autophagy is a complex multistep degradative system essential for the maintenance of cell viability, especially in post-mitotic cells as neurons and differentiated skeletal muscle cells. Interestingly, many studies concerning NDs have focused on autophagy impairment as a pathological mechanism or autophagy activity boosting to rescue the pathological phenotype. The role of VCP in autophagy has been widely debated, but recent findings have defined new mechanisms associated with VCP activity in the regulation of autophagy, showing that VCP is involved in different steps of this pathway. Here we will discuss the multiple activity of VCP in the autophagic pathway underlying its leading role either in physiological or pathological conditions. A better understanding of VCP complexes and mechanisms in regulating autophagy could define the altered mechanisms by which VCP directly or indirectly causes or modulates different human diseases and revealing possible new therapeutic approaches for NDs.
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spelling pubmed-88789542022-02-26 Valosin Containing Protein (VCP): A Multistep Regulator of Autophagy Ferrari, Veronica Cristofani, Riccardo Tedesco, Barbara Crippa, Valeria Chierichetti, Marta Casarotto, Elena Cozzi, Marta Mina, Francesco Piccolella, Margherita Galbiati, Mariarita Rusmini, Paola Poletti, Angelo Int J Mol Sci Review Valosin containing protein (VCP) has emerged as a central protein in the regulation of the protein quality control (PQC) system. VCP mutations are causative of multisystem proteinopathies, which include neurodegenerative diseases (NDs), and share various signs of altered proteostasis, mainly associated with autophagy malfunctioning. Autophagy is a complex multistep degradative system essential for the maintenance of cell viability, especially in post-mitotic cells as neurons and differentiated skeletal muscle cells. Interestingly, many studies concerning NDs have focused on autophagy impairment as a pathological mechanism or autophagy activity boosting to rescue the pathological phenotype. The role of VCP in autophagy has been widely debated, but recent findings have defined new mechanisms associated with VCP activity in the regulation of autophagy, showing that VCP is involved in different steps of this pathway. Here we will discuss the multiple activity of VCP in the autophagic pathway underlying its leading role either in physiological or pathological conditions. A better understanding of VCP complexes and mechanisms in regulating autophagy could define the altered mechanisms by which VCP directly or indirectly causes or modulates different human diseases and revealing possible new therapeutic approaches for NDs. MDPI 2022-02-09 /pmc/articles/PMC8878954/ /pubmed/35216053 http://dx.doi.org/10.3390/ijms23041939 Text en © 2022 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
Ferrari, Veronica
Cristofani, Riccardo
Tedesco, Barbara
Crippa, Valeria
Chierichetti, Marta
Casarotto, Elena
Cozzi, Marta
Mina, Francesco
Piccolella, Margherita
Galbiati, Mariarita
Rusmini, Paola
Poletti, Angelo
Valosin Containing Protein (VCP): A Multistep Regulator of Autophagy
title Valosin Containing Protein (VCP): A Multistep Regulator of Autophagy
title_full Valosin Containing Protein (VCP): A Multistep Regulator of Autophagy
title_fullStr Valosin Containing Protein (VCP): A Multistep Regulator of Autophagy
title_full_unstemmed Valosin Containing Protein (VCP): A Multistep Regulator of Autophagy
title_short Valosin Containing Protein (VCP): A Multistep Regulator of Autophagy
title_sort valosin containing protein (vcp): a multistep regulator of autophagy
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8878954/
https://www.ncbi.nlm.nih.gov/pubmed/35216053
http://dx.doi.org/10.3390/ijms23041939
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