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Condensation properties of stress granules and processing bodies are compromised in myotonic dystrophy type 1
RNA regulation in mammalian cells requires complex physical compartmentalisation, using structures thought to be formed by liquid-liquid phase separation. Disruption of these structures is implicated in numerous degenerative diseases. Myotonic dystrophy type 1 (DM1) is a multi-systemic trinucleotide...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
The Company of Biologists Ltd
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9366894/ https://www.ncbi.nlm.nih.gov/pubmed/35642886 http://dx.doi.org/10.1242/dmm.049294 |
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author | Gulyurtlu, Selma Magon, Monika S. Guest, Patrick Papavasiliou, Panagiotis P. Morrison, Kim D. Prescott, Alan R. Sleeman, Judith E. |
author_facet | Gulyurtlu, Selma Magon, Monika S. Guest, Patrick Papavasiliou, Panagiotis P. Morrison, Kim D. Prescott, Alan R. Sleeman, Judith E. |
author_sort | Gulyurtlu, Selma |
collection | PubMed |
description | RNA regulation in mammalian cells requires complex physical compartmentalisation, using structures thought to be formed by liquid-liquid phase separation. Disruption of these structures is implicated in numerous degenerative diseases. Myotonic dystrophy type 1 (DM1) is a multi-systemic trinucleotide repeat disorder resulting from an expansion of nucleotides CTG (CTGexp) in the DNA encoding DM1 protein kinase (DMPK). The cellular hallmark of DM1 is the formation of nuclear foci that contain expanded DMPK RNA (CUGexp) (with thymine instead of uracil). We report here the deregulation of stress granules (SGs) and processing bodies (P-bodies), two cytoplasmic structures key for mRNA regulation, in cell culture models of DM1. Alterations to the rates of formation and dispersal of SGs suggest an altered ability of cells to respond to stress associated with DM1, while changes to the structure and dynamics of SGs and P-bodies suggest that a widespread alteration to the biophysical properties of cellular structures is a consequence of the presence of CUGexp RNA. |
format | Online Article Text |
id | pubmed-9366894 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | The Company of Biologists Ltd |
record_format | MEDLINE/PubMed |
spelling | pubmed-93668942022-08-11 Condensation properties of stress granules and processing bodies are compromised in myotonic dystrophy type 1 Gulyurtlu, Selma Magon, Monika S. Guest, Patrick Papavasiliou, Panagiotis P. Morrison, Kim D. Prescott, Alan R. Sleeman, Judith E. Dis Model Mech Research Article RNA regulation in mammalian cells requires complex physical compartmentalisation, using structures thought to be formed by liquid-liquid phase separation. Disruption of these structures is implicated in numerous degenerative diseases. Myotonic dystrophy type 1 (DM1) is a multi-systemic trinucleotide repeat disorder resulting from an expansion of nucleotides CTG (CTGexp) in the DNA encoding DM1 protein kinase (DMPK). The cellular hallmark of DM1 is the formation of nuclear foci that contain expanded DMPK RNA (CUGexp) (with thymine instead of uracil). We report here the deregulation of stress granules (SGs) and processing bodies (P-bodies), two cytoplasmic structures key for mRNA regulation, in cell culture models of DM1. Alterations to the rates of formation and dispersal of SGs suggest an altered ability of cells to respond to stress associated with DM1, while changes to the structure and dynamics of SGs and P-bodies suggest that a widespread alteration to the biophysical properties of cellular structures is a consequence of the presence of CUGexp RNA. The Company of Biologists Ltd 2022-08-02 /pmc/articles/PMC9366894/ /pubmed/35642886 http://dx.doi.org/10.1242/dmm.049294 Text en © 2022. Published by The Company of Biologists Ltd https://creativecommons.org/licenses/by/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution and reproduction in any medium provided that the original work is properly attributed. |
spellingShingle | Research Article Gulyurtlu, Selma Magon, Monika S. Guest, Patrick Papavasiliou, Panagiotis P. Morrison, Kim D. Prescott, Alan R. Sleeman, Judith E. Condensation properties of stress granules and processing bodies are compromised in myotonic dystrophy type 1 |
title | Condensation properties of stress granules and processing bodies are compromised in myotonic dystrophy type 1 |
title_full | Condensation properties of stress granules and processing bodies are compromised in myotonic dystrophy type 1 |
title_fullStr | Condensation properties of stress granules and processing bodies are compromised in myotonic dystrophy type 1 |
title_full_unstemmed | Condensation properties of stress granules and processing bodies are compromised in myotonic dystrophy type 1 |
title_short | Condensation properties of stress granules and processing bodies are compromised in myotonic dystrophy type 1 |
title_sort | condensation properties of stress granules and processing bodies are compromised in myotonic dystrophy type 1 |
topic | Research Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9366894/ https://www.ncbi.nlm.nih.gov/pubmed/35642886 http://dx.doi.org/10.1242/dmm.049294 |
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