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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...

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Detalles Bibliográficos
Autores principales: Filadi, Riccardo, Pendin, Diana, Pizzo, Paola
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2018
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.
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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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