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Emerging Roles for Phase Separation of RNA-Binding Proteins in Cellular Pathology of ALS
Liquid-liquid phase separation (LLPS) is emerging as a major principle for the mesoscale organization of proteins, RNAs, and membrane-bound organelles into biomolecular condensates. These condensates allow for rapid cellular responses to changes in metabolic activities and signaling. Nowhere is this...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Frontiers Media S.A.
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8965147/ https://www.ncbi.nlm.nih.gov/pubmed/35372329 http://dx.doi.org/10.3389/fcell.2022.840256 |
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author | Milicevic, Katarina Rankovic, Branislava Andjus, Pavle R. Bataveljic, Danijela Milovanovic, Dragomir |
author_facet | Milicevic, Katarina Rankovic, Branislava Andjus, Pavle R. Bataveljic, Danijela Milovanovic, Dragomir |
author_sort | Milicevic, Katarina |
collection | PubMed |
description | Liquid-liquid phase separation (LLPS) is emerging as a major principle for the mesoscale organization of proteins, RNAs, and membrane-bound organelles into biomolecular condensates. These condensates allow for rapid cellular responses to changes in metabolic activities and signaling. Nowhere is this regulation more important than in neurons and glia, where cellular physiology occurs simultaneously on a range of time- and length-scales. In a number of neurodegenerative diseases, such as Amyotrophic Lateral Sclerosis (ALS), misregulation of biomolecular condensates leads to the formation of insoluble aggregates—a pathological hallmark of both sporadic and familial ALS. Here, we summarize how the emerging knowledge about the LLPS of ALS-related proteins corroborates with their aggregation. Understanding the mechanisms that lead to protein aggregation in ALS and how cells respond to these aggregates promises to open new directions for drug development. |
format | Online Article Text |
id | pubmed-8965147 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-89651472022-03-31 Emerging Roles for Phase Separation of RNA-Binding Proteins in Cellular Pathology of ALS Milicevic, Katarina Rankovic, Branislava Andjus, Pavle R. Bataveljic, Danijela Milovanovic, Dragomir Front Cell Dev Biol Cell and Developmental Biology Liquid-liquid phase separation (LLPS) is emerging as a major principle for the mesoscale organization of proteins, RNAs, and membrane-bound organelles into biomolecular condensates. These condensates allow for rapid cellular responses to changes in metabolic activities and signaling. Nowhere is this regulation more important than in neurons and glia, where cellular physiology occurs simultaneously on a range of time- and length-scales. In a number of neurodegenerative diseases, such as Amyotrophic Lateral Sclerosis (ALS), misregulation of biomolecular condensates leads to the formation of insoluble aggregates—a pathological hallmark of both sporadic and familial ALS. Here, we summarize how the emerging knowledge about the LLPS of ALS-related proteins corroborates with their aggregation. Understanding the mechanisms that lead to protein aggregation in ALS and how cells respond to these aggregates promises to open new directions for drug development. Frontiers Media S.A. 2022-02-17 /pmc/articles/PMC8965147/ /pubmed/35372329 http://dx.doi.org/10.3389/fcell.2022.840256 Text en Copyright © 2022 Milicevic, Rankovic, Andjus, Bataveljic and Milovanovic. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
spellingShingle | Cell and Developmental Biology Milicevic, Katarina Rankovic, Branislava Andjus, Pavle R. Bataveljic, Danijela Milovanovic, Dragomir Emerging Roles for Phase Separation of RNA-Binding Proteins in Cellular Pathology of ALS |
title | Emerging Roles for Phase Separation of RNA-Binding Proteins in Cellular Pathology of ALS |
title_full | Emerging Roles for Phase Separation of RNA-Binding Proteins in Cellular Pathology of ALS |
title_fullStr | Emerging Roles for Phase Separation of RNA-Binding Proteins in Cellular Pathology of ALS |
title_full_unstemmed | Emerging Roles for Phase Separation of RNA-Binding Proteins in Cellular Pathology of ALS |
title_short | Emerging Roles for Phase Separation of RNA-Binding Proteins in Cellular Pathology of ALS |
title_sort | emerging roles for phase separation of rna-binding proteins in cellular pathology of als |
topic | Cell and Developmental Biology |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8965147/ https://www.ncbi.nlm.nih.gov/pubmed/35372329 http://dx.doi.org/10.3389/fcell.2022.840256 |
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