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Mitofusin 2: from functions to disease
Mitochondria are highly dynamic organelles whose functions are essential for cell viability. Within the cell, the mitochondrial network is continuously remodeled through the balance between fusion and fission events. Moreover, it dynamically contacts other organelles, particularly the endoplasmic re...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Nature Publishing Group UK
2018
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5832425/ https://www.ncbi.nlm.nih.gov/pubmed/29491355 http://dx.doi.org/10.1038/s41419-017-0023-6 |
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author | Filadi, Riccardo Pendin, Diana Pizzo, Paola |
author_facet | Filadi, Riccardo Pendin, Diana Pizzo, Paola |
author_sort | Filadi, Riccardo |
collection | PubMed |
description | Mitochondria are highly dynamic organelles whose functions are essential for cell viability. Within the cell, the mitochondrial network is continuously remodeled through the balance between fusion and fission events. Moreover, it dynamically contacts other organelles, particularly the endoplasmic reticulum, with which it enterprises an important functional relationship able to modulate several cellular pathways. Being mitochondria key bioenergetics organelles, they have to be transported to all the specific high-energy demanding sites within the cell and, when damaged, they have to be efficiently removed. Among other proteins, Mitofusin 2 represents a key player in all these mitochondrial activities (fusion, trafficking, turnover, contacts with other organelles), the balance of which results in the appropriate mitochondrial shape, function, and distribution within the cell. Here we review the structural and functional properties of Mitofusin 2, highlighting its crucial role in several cell pathways, as well as in the pathogenesis of neurodegenerative diseases, metabolic disorders, cardiomyopathies, and cancer. |
format | Online Article Text |
id | pubmed-5832425 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | Nature Publishing Group UK |
record_format | MEDLINE/PubMed |
spelling | pubmed-58324252018-03-05 Mitofusin 2: from functions to disease Filadi, Riccardo Pendin, Diana Pizzo, Paola Cell Death Dis Review Paper Mitochondria are highly dynamic organelles whose functions are essential for cell viability. Within the cell, the mitochondrial network is continuously remodeled through the balance between fusion and fission events. Moreover, it dynamically contacts other organelles, particularly the endoplasmic reticulum, with which it enterprises an important functional relationship able to modulate several cellular pathways. Being mitochondria key bioenergetics organelles, they have to be transported to all the specific high-energy demanding sites within the cell and, when damaged, they have to be efficiently removed. Among other proteins, Mitofusin 2 represents a key player in all these mitochondrial activities (fusion, trafficking, turnover, contacts with other organelles), the balance of which results in the appropriate mitochondrial shape, function, and distribution within the cell. Here we review the structural and functional properties of Mitofusin 2, highlighting its crucial role in several cell pathways, as well as in the pathogenesis of neurodegenerative diseases, metabolic disorders, cardiomyopathies, and cancer. Nature Publishing Group UK 2018-02-28 /pmc/articles/PMC5832425/ /pubmed/29491355 http://dx.doi.org/10.1038/s41419-017-0023-6 Text en © The Author(s) 2018 Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/. |
spellingShingle | Review Paper Filadi, Riccardo Pendin, Diana Pizzo, Paola Mitofusin 2: from functions to disease |
title | Mitofusin 2: from functions to disease |
title_full | Mitofusin 2: from functions to disease |
title_fullStr | Mitofusin 2: from functions to disease |
title_full_unstemmed | Mitofusin 2: from functions to disease |
title_short | Mitofusin 2: from functions to disease |
title_sort | mitofusin 2: from functions to disease |
topic | Review Paper |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5832425/ https://www.ncbi.nlm.nih.gov/pubmed/29491355 http://dx.doi.org/10.1038/s41419-017-0023-6 |
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