Role of nucleolar dysfunction in neurodegenerative disorders: a game of genes?
Within the cell nucleus the nucleolus is the site of rRNA transcription and ribosome biogenesis and its activity is clearly essential for a correct cell function, however its specific role in neuronal homeostasis remains mainly unknown. Here we review recent evidence that impaired nucleolar activity...
| Main Authors: | , |
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| Format: | Article |
| Language: | English |
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AIMS Press
2015-05-01
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| Series: | AIMS Molecular Science |
| Subjects: | |
| Online Access: | http://www.aimspress.com/Molecular/article/313/fulltext.html |
| _version_ | 1828187663138553856 |
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| author | Rosanna Parlato Holger Bierhoff |
| author_facet | Rosanna Parlato Holger Bierhoff |
| author_sort | Rosanna Parlato |
| collection | DOAJ |
| description | Within the cell nucleus the nucleolus is the site of rRNA transcription and ribosome biogenesis and its activity is clearly essential for a correct cell function, however its specific role in neuronal homeostasis remains mainly unknown. Here we review recent evidence that impaired nucleolar activity is a common mechanism in different neurodegenerative disorders. We focus on the specific causes and consequences of impaired nucleolar activity to better understand the pathogenesis of neurodegenerative disorders, such as Alzheimer's disease (AD), Parkinson's disease (PD), Huntington's disease (HD) and amyotrophic lateral sclerosis/frontotemporal dementia (ALS/FTD). In particular, we discuss the genetic and epigenetic factors that might regulate nucleolar function in these diseases. In addition, we describe novel animal models enabling the dissection of the context-specific series of events triggered by nucleolar disruption, also known as nucleolar stress. Finally, we suggest how this novel mechanism could help to identify strategies to treat these still incurable disorders. |
| first_indexed | 2024-04-12T07:38:38Z |
| format | Article |
| id | doaj.art-31b9e1a38bef437095601d22174be369 |
| institution | Directory Open Access Journal |
| issn | 2372-0301 |
| language | English |
| last_indexed | 2024-04-12T07:38:38Z |
| publishDate | 2015-05-01 |
| publisher | AIMS Press |
| record_format | Article |
| series | AIMS Molecular Science |
| spelling | doaj.art-31b9e1a38bef437095601d22174be3692022-12-22T03:41:52ZengAIMS PressAIMS Molecular Science2372-03012015-05-012321122410.3934/molsci.2015.3.211201503211Role of nucleolar dysfunction in neurodegenerative disorders: a game of genes?Rosanna ParlatoHolger Bierhoff0Dept. of Molecular Biology of the Cell II, DKFZ-ZMBH Alliance, German Cancer Research Center, Heidelberg, GermanWithin the cell nucleus the nucleolus is the site of rRNA transcription and ribosome biogenesis and its activity is clearly essential for a correct cell function, however its specific role in neuronal homeostasis remains mainly unknown. Here we review recent evidence that impaired nucleolar activity is a common mechanism in different neurodegenerative disorders. We focus on the specific causes and consequences of impaired nucleolar activity to better understand the pathogenesis of neurodegenerative disorders, such as Alzheimer's disease (AD), Parkinson's disease (PD), Huntington's disease (HD) and amyotrophic lateral sclerosis/frontotemporal dementia (ALS/FTD). In particular, we discuss the genetic and epigenetic factors that might regulate nucleolar function in these diseases. In addition, we describe novel animal models enabling the dissection of the context-specific series of events triggered by nucleolar disruption, also known as nucleolar stress. Finally, we suggest how this novel mechanism could help to identify strategies to treat these still incurable disorders.http://www.aimspress.com/Molecular/article/313/fulltext.htmlnucleoluscellular stressrRNAneurodegenerationmouse modelsepigenetic regulation |
| spellingShingle | Rosanna Parlato Holger Bierhoff Role of nucleolar dysfunction in neurodegenerative disorders: a game of genes? AIMS Molecular Science nucleolus cellular stress rRNA neurodegeneration mouse models epigenetic regulation |
| title | Role of nucleolar dysfunction in neurodegenerative disorders: a game of genes? |
| title_full | Role of nucleolar dysfunction in neurodegenerative disorders: a game of genes? |
| title_fullStr | Role of nucleolar dysfunction in neurodegenerative disorders: a game of genes? |
| title_full_unstemmed | Role of nucleolar dysfunction in neurodegenerative disorders: a game of genes? |
| title_short | Role of nucleolar dysfunction in neurodegenerative disorders: a game of genes? |
| title_sort | role of nucleolar dysfunction in neurodegenerative disorders a game of genes |
| topic | nucleolus cellular stress rRNA neurodegeneration mouse models epigenetic regulation |
| url | http://www.aimspress.com/Molecular/article/313/fulltext.html |
| work_keys_str_mv | AT rosannaparlato roleofnucleolardysfunctioninneurodegenerativedisordersagameofgenes AT holgerbierhoff roleofnucleolardysfunctioninneurodegenerativedisordersagameofgenes |