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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...

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Detalles Bibliográficos
Autores principales: Reddy, Sita, Comai, Lucio
Formato: Online Artículo Texto
Lenguaje:English
Publicado: F1000Research 2016
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.
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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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