Repeat-associated non-AUG (RAN) translation generates toxic repeat proteins from pathological repeat expansions found in certain neurodegenerative disorders, including amyotrophic lateral sclerosis and frontotemporal dementia. How to suppress RAN translation has so far been unknown.
Saskia Hutten, Dorothee Dormann
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Identification of Novel Modifiers of RAN Translation at FMR1 and C9ORF72
2021Nucleotide-repeat expansions (NREs) underlie a heterogeneous group of neurodegenerative and neuromuscular disorders for which there are currently no effective therapies. Recently, it was discovered that NREs in mRNA can support translation initiation in the absence of an AUG start codon across a wide variety of sequence contexts, and that the products ...
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Length-dependent RNA foci formation and RAN translation are associated with SCA12 disorder
2023Summary Spinocerebellar ataxia type-12 (SCA-12) is a neurodegenerative disease caused by tandem CAG repeat expansion in the 5’-UTR/non-coding region of PPP2R2B . Molecular pathology of SCA12 has not been studied in the context of CAG repeats and no appropriate models exist. We found
Manish Kumar +8 more
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Post-translational lysine-acetylation of Ran and its regulation by Sirtuin deacetylases [PDF]
, 2016 Through recent advances in high-throughput mass spectrometry it has become evident that post-translational N-(epsilon)-lysine-acetylation is a modification found on thousands of proteins of all cellular compartments and all essential physiological processes.
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Investigation of RAN Translation in SCA2 (P05.043)
Neurology, 2013Lance Petersen +3 more
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Mechanistic studies on the regulation of Ran-function by post-translational lysine-acetylation [PDF]
, 2015 The small GTP-binding protein Ran regulates fundamental cellular processes such as nucleocytoplasmic transport, nuclear envelope formation and mitotic spindle assembly. The two nucleotide states of Ran, GTP- and GDP-bound, are characterized by distinct conformations of two regulatory regions (switch I and switch II) which determine effector binding ...
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Translation of “当然(Dang Ran)” and “Of Course” in Energy Texts
Modern Linguistics, 2022openaire +1 more source
Metformin inhibits RAN translation through PKR pathway and mitigates disease in C9orf72 ALS/FTD mice
Proceedings of the National Academy of Sciences of the United States of America, 2020Tao Zu +2 more
exaly
O07 RAN translation of expanded CGG repeat in LRP12 may contribute to oculopharyngodistal myopathy
Neuromuscular Disorders, 2023C. Li +4 more
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INV08 RAN translation in C9orf72 ALS/FTD and other repeat opportunities
Neuromuscular Disorders, 2023openaire +1 more source