Dysregulated axonal RNA translation in amyotrophic lateral sclerosis.
作者： Kyota Yasuda , Stavroula Mili
DOI: 10.1002/WRNA.1352
关键词:
摘要: Amyotrophic lateral sclerosis (ALS) is an adult-onset motor neuron disease that has been associated with a diverse array of genetic changes. Prominent among these are mutations in RNA-binding proteins (RBPs) or repeat expansions give rise to toxic RNA species. RBPs additionally central components pathologic aggregates constitute hallmark, suggesting dysregulation metabolism underlies progression. In the context neuronal physiology, transport RNAs and localized translation axons fundamental survival function. Several lines evidence suggest axonal process perturbed by various pathogenic events ALS. Dysregulated specific groups could underlie feedback effects connect reinforce manifestations. Among such candidates encoding involved regulation microtubule dynamics. Further understanding axonally dysregulated targets mechanisms they induce provide useful therapeutic insights. WIREs 2016, 7:589-603. doi: 10.1002/wrna.1352 For further resources related this article, please visit website.
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