The Interplay Between Autophagy and RNA Homeostasis: Implications for Amyotrophic Lateral Sclerosis and Frontotemporal Dementia
Amyotrophic lateral sclerosis and frontotemporal dementia are neurodegenerative disorders that lie on a disease spectrum, sharing genetic causes and pathology, and both without effective therapeutics. Two pathways that have been shown to play major roles in disease pathogenesis are autophagy and RNA...
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Frontiers Media S.A.
2022-04-01
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| Series: | Frontiers in Cell and Developmental Biology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fcell.2022.838402/full |
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| author | O. H. Houghton O. H. Houghton S. Mizielinska S. Mizielinska P. Gomez-Suaga P. Gomez-Suaga P. Gomez-Suaga P. Gomez-Suaga |
| author_facet | O. H. Houghton O. H. Houghton S. Mizielinska S. Mizielinska P. Gomez-Suaga P. Gomez-Suaga P. Gomez-Suaga P. Gomez-Suaga |
| author_sort | O. H. Houghton |
| collection | DOAJ |
| description | Amyotrophic lateral sclerosis and frontotemporal dementia are neurodegenerative disorders that lie on a disease spectrum, sharing genetic causes and pathology, and both without effective therapeutics. Two pathways that have been shown to play major roles in disease pathogenesis are autophagy and RNA homeostasis. Intriguingly, there is an increasing body of evidence suggesting a critical interplay between these pathways. Autophagy is a multi-stage process for bulk and selective clearance of malfunctional cellular components, with many layers of regulation. Although the majority of autophagy research focuses on protein degradation, it can also mediate RNA catabolism. ALS/FTD-associated proteins are involved in many stages of autophagy and autophagy-mediated RNA degradation, particularly converging on the clearance of persistent pathological stress granules. In this review, we will summarise the progress in understanding the autophagy-RNA homeostasis interplay and how that knowledge contributes to our understanding of the pathobiology of ALS/FTD. |
| first_indexed | 2024-04-13T03:43:31Z |
| format | Article |
| id | doaj.art-55929b44047c46b6aa6a4ab7732d685a |
| institution | Directory Open Access Journal |
| issn | 2296-634X |
| language | English |
| last_indexed | 2024-04-13T03:43:31Z |
| publishDate | 2022-04-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Cell and Developmental Biology |
| spelling | doaj.art-55929b44047c46b6aa6a4ab7732d685a2022-12-22T03:04:05ZengFrontiers Media S.A.Frontiers in Cell and Developmental Biology2296-634X2022-04-011010.3389/fcell.2022.838402838402The Interplay Between Autophagy and RNA Homeostasis: Implications for Amyotrophic Lateral Sclerosis and Frontotemporal DementiaO. H. Houghton0O. H. Houghton1S. Mizielinska2S. Mizielinska3P. Gomez-Suaga4P. Gomez-Suaga5P. Gomez-Suaga6P. Gomez-Suaga7Department of Basic and Clinical Neuroscience, Institute of Psychiatry, Psychology and Neuroscience, King’s College London, Maurice Wohl Clinical Neuroscience Institute, London, United KingdomUK Dementia Research Institute at King’s College London, London, United KingdomDepartment of Basic and Clinical Neuroscience, Institute of Psychiatry, Psychology and Neuroscience, King’s College London, Maurice Wohl Clinical Neuroscience Institute, London, United KingdomUK Dementia Research Institute at King’s College London, London, United KingdomDepartment of Basic and Clinical Neuroscience, Institute of Psychiatry, Psychology and Neuroscience, King’s College London, Maurice Wohl Clinical Neuroscience Institute, London, United KingdomDepartamento de Bioquímica y Biología Molecular y Genética, Facultad de Enfermería y Terapia Ocupacional, Universidad de Extremadura, Cáceres, SpainCentro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (CIBERNED), Madrid, SpainInstituto Universitario de Investigación Biosanitaria de Extremadura (INUBE), Cáceres, SpainAmyotrophic lateral sclerosis and frontotemporal dementia are neurodegenerative disorders that lie on a disease spectrum, sharing genetic causes and pathology, and both without effective therapeutics. Two pathways that have been shown to play major roles in disease pathogenesis are autophagy and RNA homeostasis. Intriguingly, there is an increasing body of evidence suggesting a critical interplay between these pathways. Autophagy is a multi-stage process for bulk and selective clearance of malfunctional cellular components, with many layers of regulation. Although the majority of autophagy research focuses on protein degradation, it can also mediate RNA catabolism. ALS/FTD-associated proteins are involved in many stages of autophagy and autophagy-mediated RNA degradation, particularly converging on the clearance of persistent pathological stress granules. In this review, we will summarise the progress in understanding the autophagy-RNA homeostasis interplay and how that knowledge contributes to our understanding of the pathobiology of ALS/FTD.https://www.frontiersin.org/articles/10.3389/fcell.2022.838402/fullautophagyRNAamyotrophic lateral sclerosisfrontotemporal dementiaRNA-binding proteinsC9orf72 |
| spellingShingle | O. H. Houghton O. H. Houghton S. Mizielinska S. Mizielinska P. Gomez-Suaga P. Gomez-Suaga P. Gomez-Suaga P. Gomez-Suaga The Interplay Between Autophagy and RNA Homeostasis: Implications for Amyotrophic Lateral Sclerosis and Frontotemporal Dementia Frontiers in Cell and Developmental Biology autophagy RNA amyotrophic lateral sclerosis frontotemporal dementia RNA-binding proteins C9orf72 |
| title | The Interplay Between Autophagy and RNA Homeostasis: Implications for Amyotrophic Lateral Sclerosis and Frontotemporal Dementia |
| title_full | The Interplay Between Autophagy and RNA Homeostasis: Implications for Amyotrophic Lateral Sclerosis and Frontotemporal Dementia |
| title_fullStr | The Interplay Between Autophagy and RNA Homeostasis: Implications for Amyotrophic Lateral Sclerosis and Frontotemporal Dementia |
| title_full_unstemmed | The Interplay Between Autophagy and RNA Homeostasis: Implications for Amyotrophic Lateral Sclerosis and Frontotemporal Dementia |
| title_short | The Interplay Between Autophagy and RNA Homeostasis: Implications for Amyotrophic Lateral Sclerosis and Frontotemporal Dementia |
| title_sort | interplay between autophagy and rna homeostasis implications for amyotrophic lateral sclerosis and frontotemporal dementia |
| topic | autophagy RNA amyotrophic lateral sclerosis frontotemporal dementia RNA-binding proteins C9orf72 |
| url | https://www.frontiersin.org/articles/10.3389/fcell.2022.838402/full |
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