Titlebook: RNA Metabolism in Neurodegenerative Diseases; Rita Sattler,Christopher J. Donnelly Book 2018 Springer International Publishing AG, part of

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RNA Nucleocytoplasmic Transport Defects in Neurodegenerative Diseases,s into the cytoplasm. This is accomplished by a variety of transport complexes that contain either a member of the exportin family of proteins and translocation fueled by GTP hydrolysis or in the case of mRNA by complexes containing the export protein NXF1. Recent evidence indicates that RNA transpo

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Senataxin, A Novel Helicase at the Interface of RNA Transcriptome Regulation and Neurobiology: Fromave established the importance of SETX for neural function, as recessive mutations in the . gene cause Ataxia with Oculomotor Apraxia type 2 (AOA2) (OMIM: 606002), which is the third most common form of recessive ataxia, after Friedreich’s ataxia and Ataxia-Telangiectasia. In addition, rare, dominan

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Lost in Translation: Evidence for Protein Synthesis Deficits in ALS/FTD and Related Neurodegeneratiit. Although the complexity provides robust regulation of proteostasis, it also offers several opportunities for translational dysregulation, as has been observed in many neurodegenerative disorders. Defective mRNA localization, mRNA sequatration, inhibited ribogenesis, mutant tRNA synthetases, and