Journal
PROGRESS IN NEUROBIOLOGY
Volume 97, Issue 2, Pages 173-189Publisher
PERGAMON-ELSEVIER SCIENCE LTD
DOI: 10.1016/j.pneurobio.2011.10.006
Keywords
miRNA; TDP-43; Fragile X; Bidirectional transcription; CAG RNA; Repeat expansion disorders
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Funding
- NIH [K08NS069809]
- Bucky and Patti Harris Professorship
- Systems and Integrative Biology Training Grant
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The expression, processing, transport and activities of both coding and non-coding RNAs play critical roles in normal neuronal function and differentiation. Over the past decade, these same pathways have come under scrutiny as potential contributors to neurodegenerative disease. Here we focus broadly on the roles of RNA and RNA processing in neurodegeneration. We first discuss a set of RNAopathies, where non-coding repeat expansions drive pathogenesis through a surprisingly diverse set of mechanisms. We next explore an emerging class of RNA binding proteinopathies where redistribution and aggregation of the RNA binding proteins TDP-43 or FUS contribute to a potentially broad range of neurodegenerative disorders. Lastly, we delve into the potential contributions of alterations in both short and long non-coding RNAs to neurodegenerative illness. (C) 2011 Elsevier Ltd. All rights reserved.
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