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The nuclear lamina in health and disease

The nuclear lamina (NL) is a structural component of the nuclear envelope and makes extensive contacts with integral nuclear membrane proteins and chromatin. These interactions are critical for many cellular processes, such as nuclear positioning, perception of mechanical stimuli from the cell surfa...

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Autores principales: Dobrzynska, Agnieszka, Gonzalo, Susana, Shanahan, Catherine, Askjaer, Peter
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Taylor & Francis 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4991244/
https://www.ncbi.nlm.nih.gov/pubmed/27158763
http://dx.doi.org/10.1080/19491034.2016.1183848
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author Dobrzynska, Agnieszka
Gonzalo, Susana
Shanahan, Catherine
Askjaer, Peter
author_facet Dobrzynska, Agnieszka
Gonzalo, Susana
Shanahan, Catherine
Askjaer, Peter
author_sort Dobrzynska, Agnieszka
collection PubMed
description The nuclear lamina (NL) is a structural component of the nuclear envelope and makes extensive contacts with integral nuclear membrane proteins and chromatin. These interactions are critical for many cellular processes, such as nuclear positioning, perception of mechanical stimuli from the cell surface, nuclear stability, 3-dimensional organization of chromatin and regulation of chromatin-binding proteins, including transcription factors. The NL is present in all nucleated metazoan cells but its composition and interactome differ between tissues. Most likely, this contributes to the broad spectrum of disease manifestations in humans with mutations in NL-related genes, ranging from muscle dystrophies to neurological disorders, lipodystrophies and progeria syndromes. We review here exciting novel insight into NL function at the cellular level, in particular in chromatin organization and mechanosensation. We also present recent observations on the relation between the NL and metabolism and the special relevance of the NL in muscle tissues. Finally, we discuss new therapeutic approaches to treat NL-related diseases.
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spelling pubmed-49912442016-08-30 The nuclear lamina in health and disease Dobrzynska, Agnieszka Gonzalo, Susana Shanahan, Catherine Askjaer, Peter Nucleus Commentary The nuclear lamina (NL) is a structural component of the nuclear envelope and makes extensive contacts with integral nuclear membrane proteins and chromatin. These interactions are critical for many cellular processes, such as nuclear positioning, perception of mechanical stimuli from the cell surface, nuclear stability, 3-dimensional organization of chromatin and regulation of chromatin-binding proteins, including transcription factors. The NL is present in all nucleated metazoan cells but its composition and interactome differ between tissues. Most likely, this contributes to the broad spectrum of disease manifestations in humans with mutations in NL-related genes, ranging from muscle dystrophies to neurological disorders, lipodystrophies and progeria syndromes. We review here exciting novel insight into NL function at the cellular level, in particular in chromatin organization and mechanosensation. We also present recent observations on the relation between the NL and metabolism and the special relevance of the NL in muscle tissues. Finally, we discuss new therapeutic approaches to treat NL-related diseases. Taylor & Francis 2016-05-09 /pmc/articles/PMC4991244/ /pubmed/27158763 http://dx.doi.org/10.1080/19491034.2016.1183848 Text en © 2016 The Author(s). Published with license by Taylor & Francis Group, LLC http://creativecommons.org/licenses/by-nc/3.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution-Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/), which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. The moral rights of the named author(s) have been asserted.
spellingShingle Commentary
Dobrzynska, Agnieszka
Gonzalo, Susana
Shanahan, Catherine
Askjaer, Peter
The nuclear lamina in health and disease
title The nuclear lamina in health and disease
title_full The nuclear lamina in health and disease
title_fullStr The nuclear lamina in health and disease
title_full_unstemmed The nuclear lamina in health and disease
title_short The nuclear lamina in health and disease
title_sort nuclear lamina in health and disease
topic Commentary
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4991244/
https://www.ncbi.nlm.nih.gov/pubmed/27158763
http://dx.doi.org/10.1080/19491034.2016.1183848
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