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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...
Autores principales: | , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2022
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Materias: | |
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. |
format | Online Article Text |
id | pubmed-8878954 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
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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