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Recent advances in understanding the role of lamins in health and disease
Lamins are major components of the nuclear lamina, a network of proteins that supports the nuclear envelope in metazoan cells. Over the past decade, biochemical studies have provided support for the view that lamins are not passive bystanders providing mechanical stability to the nucleus but play an...
Autores principales: | , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
F1000Research
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5074355/ https://www.ncbi.nlm.nih.gov/pubmed/27803806 http://dx.doi.org/10.12688/f1000research.9260.1 |
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author | Reddy, Sita Comai, Lucio |
author_facet | Reddy, Sita Comai, Lucio |
author_sort | Reddy, Sita |
collection | PubMed |
description | Lamins are major components of the nuclear lamina, a network of proteins that supports the nuclear envelope in metazoan cells. Over the past decade, biochemical studies have provided support for the view that lamins are not passive bystanders providing mechanical stability to the nucleus but play an active role in the organization of the genome and the function of fundamental nuclear processes. It has also become apparent that lamins are critical for human health, as a large number of mutations identified in the gene that encodes for A-type lamins are associated with tissue-specific and systemic genetic diseases, including the accelerated aging disorder known as Hutchinson-Gilford progeria syndrome. Recent years have witnessed great advances in our understanding of the role of lamins in the nucleus and the functional consequences of disease-associated A-type lamin mutations. Many of these findings have been presented in comprehensive reviews. In this mini-review, we discuss recent breakthroughs in the role of lamins in health and disease and what lies ahead in lamin research. |
format | Online Article Text |
id | pubmed-5074355 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2016 |
publisher | F1000Research |
record_format | MEDLINE/PubMed |
spelling | pubmed-50743552016-10-31 Recent advances in understanding the role of lamins in health and disease Reddy, Sita Comai, Lucio F1000Res Review Lamins are major components of the nuclear lamina, a network of proteins that supports the nuclear envelope in metazoan cells. Over the past decade, biochemical studies have provided support for the view that lamins are not passive bystanders providing mechanical stability to the nucleus but play an active role in the organization of the genome and the function of fundamental nuclear processes. It has also become apparent that lamins are critical for human health, as a large number of mutations identified in the gene that encodes for A-type lamins are associated with tissue-specific and systemic genetic diseases, including the accelerated aging disorder known as Hutchinson-Gilford progeria syndrome. Recent years have witnessed great advances in our understanding of the role of lamins in the nucleus and the functional consequences of disease-associated A-type lamin mutations. Many of these findings have been presented in comprehensive reviews. In this mini-review, we discuss recent breakthroughs in the role of lamins in health and disease and what lies ahead in lamin research. F1000Research 2016-10-19 /pmc/articles/PMC5074355/ /pubmed/27803806 http://dx.doi.org/10.12688/f1000research.9260.1 Text en Copyright: © 2016 Reddy S and Comai L http://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the terms of the Creative Commons Attribution Licence, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Review Reddy, Sita Comai, Lucio Recent advances in understanding the role of lamins in health and disease |
title | Recent advances in understanding the role of lamins in health and disease |
title_full | Recent advances in understanding the role of lamins in health and disease |
title_fullStr | Recent advances in understanding the role of lamins in health and disease |
title_full_unstemmed | Recent advances in understanding the role of lamins in health and disease |
title_short | Recent advances in understanding the role of lamins in health and disease |
title_sort | recent advances in understanding the role of lamins in health and disease |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5074355/ https://www.ncbi.nlm.nih.gov/pubmed/27803806 http://dx.doi.org/10.12688/f1000research.9260.1 |
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