Abstract:
:A novel RNA-mediated disease mechanism has emerged from studies on dominantly inherited neurological disorders caused by unstable microsatellite expansions in non-coding regions of the genome. These non-coding tandem repeat expansions trigger the production of unusual RNAs that gain a toxic function, which involves the formation of RNA repeat structures that interact with, and alter the activities of, various factors required for normal RNA processing as well as additional cellular functions. In this review, we explore the deleterious effects of toxic RNA expression and discuss the various model systems currently available for studying RNA gain-of-function in neurologic diseases. Common themes, including bidirectional transcription and repeat-associated non-ATG (RAN) translation, have recently emerged from expansion disease studies. These and other discoveries have highlighted the need for further investigations designed to provide the additional mechanistic insights essential for future therapeutic development.
journal_name
Brain Resjournal_title
Brain researchauthors
Mohan A,Goodwin M,Swanson MSdoi
10.1016/j.brainres.2014.03.039subject
Has Abstractpub_date
2014-10-10 00:00:00pages
3-14eissn
0006-8993issn
1872-6240pii
S0006-8993(14)00417-Xjournal_volume
1584pub_type
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