RNA metabolism in neurodegenerative disease

Aging-related neurodegenerative diseases are progressive and fatal neurological diseases that are characterized by irreversible neuron loss and gliosis. With a growing population of aging individuals, there is a pressing need to better understand the basic biology underlying these diseases. Although...

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Bibliographic Details
Published in:Disease models & mechanisms 2017-05, Vol.10 (5), p.509-518
Main Authors: Liu, Elaine Y, Cali, Christopher P, Lee, Edward B
Format: Article
Language:English
Subjects:
Alzheimer's disease
Amyotrophic lateral sclerosis
Animals
At A Glance
Autophagy
Cytoplasm - metabolism
Dementia
Disease
Gene expression
Humans
Hydrogels
lncRNA
Metabolism
MicroRNAs
MicroRNAs - genetics
Microsatellite Repeats
miRNA
Mutation
Neurodegeneration
Neurodegenerative Diseases - genetics
Neurodegenerative Diseases - metabolism
Neurons
Pathogenesis
Proteins
RNA
RNA - metabolism
RNA binding proteins
RNA Processing, Post-Transcriptional
RNA, Untranslated - genetics
RNA-Binding Proteins - metabolism
Toxicity
tRNA
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Summary:Aging-related neurodegenerative diseases are progressive and fatal neurological diseases that are characterized by irreversible neuron loss and gliosis. With a growing population of aging individuals, there is a pressing need to better understand the basic biology underlying these diseases. Although diverse disease mechanisms have been implicated in neurodegeneration, a common theme of altered RNA processing has emerged as a unifying contributing factor to neurodegenerative disease. RNA processing includes a series of distinct processes, including RNA splicing, transport and stability, as well as the biogenesis of non-coding RNAs. Here, we highlight how some of these mechanisms are altered in neurodegenerative disease, including the mislocalization of RNA-binding proteins and their sequestration induced by microsatellite repeats, microRNA biogenesis alterations and defective tRNA biogenesis, as well as changes to long-intergenic non-coding RNAs. We also highlight potential therapeutic interventions for each of these mechanisms.
ISSN:1754-8403
1754-8411
DOI:10.1242/dmm.028613