| 书目名称 | RNA Metabolism in Neurodegenerative Diseases | | 编辑 | Rita Sattler,Christopher J. Donnelly | | 视频video | | | 概述 | Looks at major neurodegenerative diseases affected by RNA toxicity.Explores multiple disease mechanisms triggered by RNA metabolism.Taken together, the chapters provide insight on potential therapeuti | | 丛书名称 | Advances in Neurobiology | | 图书封面 |  | | 描述 | .It has become evident over the last years that abnormalities in RNA processing play a fundamental part in the pathogenesis of neurodegenerative diseases. Cellular viability depends on proper regulation of RNA metabolism and subsequent protein synthesis, which requires the interplay of many processes including transcription, pre--‐mRNA splicing, mRNA editing as well as mRNA stability, transport and translation. Dysfunction in any of these processes, often caused by mutations in the coding and non--‐ coding RNAs, can be very destructive to the cellular environment and consequently impair neural viability. The result of this RNA toxicity can lead to a toxic gain of function or a loss of function, depending on the nature of the mutation. For example, in repeat expansion disorders, such as the newly discovered hexanucleotide repeat expansion in the.C9orf72 .gene found in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), a toxic gain of function leads to the formation of RNA foci and the sequestration of RNA binding proteins (RBPs). This in return leads to a loss of function of those RBPs, which is hypothesized to play a significant part in the disease progression | | 出版日期 | Book 2018 | | 关键词 | RNA metabolism; RNA toxicity; neurodegeneration; RNA processing; amyotrophic lateral sclerosis (ALS) | | 版次 | 1 | | doi | https://doi.org/10.1007/978-3-319-89689-2 | | isbn_softcover | 978-3-030-07832-4 | | isbn_ebook | 978-3-319-89689-2Series ISSN 2190-5215 Series E-ISSN 2190-5223 | | issn_series | 2190-5215 | | copyright | Springer International Publishing AG, part of Springer Nature 2018 |
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发表于 2025-3-21 19:15:35
