RNA metabolism in neurodegenerative disease

• Main Authors: Elaine Y. Liu (Author), Christopher P. Cali (Author), Edward B. Lee (Author)
• Format: Book
• Published: The Company of Biologists, 2017-05-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.
• Notes: 1754-8403; 1754-8411; 10.1242/dmm.028613
• Keywords: Disease; RNA binding proteins; Microsatellite repeats; miRNA; tRNA; lncRNA; RNA; Medicine; R; Pathology; RB1-214
• Source: Disease Models & Mechanisms, Vol 10, Iss 5, Pp 509-518 (2017)

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.