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Structural and Mechanical Aberrations of the Nuclear Lamina in Disease

The nuclear lamins are the major components of the nuclear lamina in the nuclear envelope. Lamins are involved in numerous functions, including a role in providing structural support to the cell and the mechanosensing of the cell. Mutations in the genes encoding for lamins lead to the rare diseases...

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Autores principales: Stiekema, Merel, van Zandvoort, Marc A. M. J., Ramaekers, Frans C. S., Broers, Jos L. V.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7464082/
https://www.ncbi.nlm.nih.gov/pubmed/32796718
http://dx.doi.org/10.3390/cells9081884
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author Stiekema, Merel
van Zandvoort, Marc A. M. J.
Ramaekers, Frans C. S.
Broers, Jos L. V.
author_facet Stiekema, Merel
van Zandvoort, Marc A. M. J.
Ramaekers, Frans C. S.
Broers, Jos L. V.
author_sort Stiekema, Merel
collection PubMed
description The nuclear lamins are the major components of the nuclear lamina in the nuclear envelope. Lamins are involved in numerous functions, including a role in providing structural support to the cell and the mechanosensing of the cell. Mutations in the genes encoding for lamins lead to the rare diseases termed laminopathies. However, not only laminopathies show alterations in the nuclear lamina. Deregulation of lamin expression is reported in multiple cancers and several viral infections lead to a disrupted nuclear lamina. The structural and mechanical effects of alterations in the nuclear lamina can partly explain the phenotypes seen in disease, such as muscular weakness in certain laminopathies and transmigration of cancer cells. However, a lot of answers to questions about the relation between changes in the nuclear lamina and disease development remain elusive. Here, we review the current understandings of the contribution of the nuclear lamina in the structural support and mechanosensing of healthy and diseased cells.
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spelling pubmed-74640822020-09-04 Structural and Mechanical Aberrations of the Nuclear Lamina in Disease Stiekema, Merel van Zandvoort, Marc A. M. J. Ramaekers, Frans C. S. Broers, Jos L. V. Cells Review The nuclear lamins are the major components of the nuclear lamina in the nuclear envelope. Lamins are involved in numerous functions, including a role in providing structural support to the cell and the mechanosensing of the cell. Mutations in the genes encoding for lamins lead to the rare diseases termed laminopathies. However, not only laminopathies show alterations in the nuclear lamina. Deregulation of lamin expression is reported in multiple cancers and several viral infections lead to a disrupted nuclear lamina. The structural and mechanical effects of alterations in the nuclear lamina can partly explain the phenotypes seen in disease, such as muscular weakness in certain laminopathies and transmigration of cancer cells. However, a lot of answers to questions about the relation between changes in the nuclear lamina and disease development remain elusive. Here, we review the current understandings of the contribution of the nuclear lamina in the structural support and mechanosensing of healthy and diseased cells. MDPI 2020-08-11 /pmc/articles/PMC7464082/ /pubmed/32796718 http://dx.doi.org/10.3390/cells9081884 Text en © 2020 by the authors. 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 (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Stiekema, Merel
van Zandvoort, Marc A. M. J.
Ramaekers, Frans C. S.
Broers, Jos L. V.
Structural and Mechanical Aberrations of the Nuclear Lamina in Disease
title Structural and Mechanical Aberrations of the Nuclear Lamina in Disease
title_full Structural and Mechanical Aberrations of the Nuclear Lamina in Disease
title_fullStr Structural and Mechanical Aberrations of the Nuclear Lamina in Disease
title_full_unstemmed Structural and Mechanical Aberrations of the Nuclear Lamina in Disease
title_short Structural and Mechanical Aberrations of the Nuclear Lamina in Disease
title_sort structural and mechanical aberrations of the nuclear lamina in disease
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7464082/
https://www.ncbi.nlm.nih.gov/pubmed/32796718
http://dx.doi.org/10.3390/cells9081884
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