RNA metabolism in neurodegenerative disease

• Main Authors: Elaine Y. Liu, Christopher P. Cali, Edward B. Lee
• Format: Article
• Language: English
• Published: The Company of Biologists 2017-05-01
• Series: Disease Models & Mechanisms
• Subjects: Disease; RNA binding proteins; Microsatellite repeats; miRNA; tRNA; lncRNA; RNA
• Online Access: http://dmm.biologists.org/content/10/5/509
• ISSN: 1754-8403; 1754-8411

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.