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Prominent among these are mutations in <jats:styled-content style=\"fixed-case\">RNA<\/jats:styled-content>\u2010binding proteins (<jats:styled-content style=\"fixed-case\">RBPs<\/jats:styled-content>) or repeat expansions that give rise to toxic <jats:styled-content style=\"fixed-case\">RNA<\/jats:styled-content> species. <jats:styled-content style=\"fixed-case\">RBPs<\/jats:styled-content> are additionally central components of pathologic aggregates that constitute a disease hallmark, suggesting that dysregulation of <jats:styled-content style=\"fixed-case\">RNA<\/jats:styled-content> metabolism underlies disease progression. In the context of neuronal physiology, transport of <jats:styled-content style=\"fixed-case\">RNAs<\/jats:styled-content> and localized <jats:styled-content style=\"fixed-case\">RNA<\/jats:styled-content> translation in axons are fundamental to neuronal survival and function. Several lines of evidence suggest that axonal <jats:styled-content style=\"fixed-case\">RNA<\/jats:styled-content> translation is a central process perturbed by various pathogenic events associated with <jats:styled-content style=\"fixed-case\">ALS<\/jats:styled-content>. Dysregulated translation of specific <jats:styled-content style=\"fixed-case\">RNA<\/jats:styled-content> groups could underlie feedback effects that connect and reinforce disease manifestations. Among such candidates are <jats:styled-content style=\"fixed-case\">RNAs<\/jats:styled-content> encoding proteins involved in the regulation of microtubule dynamics. Further understanding of axonally dysregulated <jats:styled-content style=\"fixed-case\">RNA<\/jats:styled-content> targets and of the feedback mechanisms they induce could provide useful therapeutic insights. <jats:italic>WIREs RNA<\/jats:italic> 2016, 7:589\u2013603. doi: 10.1002\/wrna.1352<\/jats:p><jats:p>This article is categorized under:\n<jats:list list-type=\"explicit-label\">\n<jats:list-item><jats:p>Translation &gt; Translation Regulation<\/jats:p><\/jats:list-item>\n<jats:list-item><jats:p>RNA Export and Localization &gt; RNA Localization<\/jats:p><\/jats:list-item>\n<jats:list-item><jats:p>RNA in Disease and Development &gt; RNA in Disease<\/jats:p><\/jats:list-item>\n<\/jats:list><\/jats:p>","DOI":"10.1002\/wrna.1352","type":"journal-article","created":{"date-parts":[[2016,4,3]],"date-time":"2016-04-03T16:30:03Z","timestamp":1459701003000},"page":"589-603","source":"Crossref","is-referenced-by-count":23,"title":["Dysregulated axonal <scp>RNA<\/scp> translation in 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