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Title: | Emerging Roles for Phase Separation of RNA-Binding Proteins in Cellular Pathology of ALS | Authors: | Milićević, Katarina Rankovic, Branislava Anđus, Pavle Bataveljić B. Danijela Milovanović Dragomir |
Keywords: | Liquid-liquid phase separation;FUS, TDP-43;RNP aggregates;Stress granule;Neurons;Amyotrophic lateral sclerosis | Issue Date: | 17-Feb-2022 | Rank: | M21 | Publisher: | National Library of Medicine | Citation: | Milicevic K, Rankovic B, Andjus PR, Bataveljic D, Milovanovic D. Emerging Roles for Phase Separation of RNA-Binding Proteins in Cellular Pathology of ALS. Front Cell Dev Biol. 2022 Feb 17;10:840256. doi: 10.3389/fcell.2022.840256. PMID: 35372329; PMCID: PMC8965147. | Project: | Ministry of Education, Science and Technological Development of Republic of Serbia bilateral project with Federal Republic of Germany (2021/2022 to DB; Contract No. 451-03-9/2021-14/200178). | Journal: | Frontiers in Cell and Developmental Biology | Abstract: | 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. |
URI: | https://biore.bio.bg.ac.rs/handle/123456789/4998 | ISSN: | 2296-634X | DOI: | 10.3389/fcell.2022.840256 |
Appears in Collections: | Journal Article |
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