Non-ATG–initiated translation directed by microsatellite expansions

By T. Zu, Brian B. Gibbens, Noelle S. Doty, M. Gomes-Pereira, A. Huguet, Matthew D. Stone, Jamie M. Margolis, M. Peterson, Todd W Markowski, Melissa A. C. Ingram and 12 more. Proceedings of the National Academy of Sciences of the United States of America, 2010

858 citations recorded by the source corpus.

Subfields: Cellular and Molecular Neuroscience, Molecular Biology

View Paper: https://www.semanticscholar.org/paper/3796b3993a5474e97c364bf7a9b76fb3a50389c5

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Citations in the corpus

Unconventional translation of C9ORF72 GGGGCC expansion generates insoluble polypeptides specific to c9FTD/ALS. (2013, positive): “Given evidence that RAN translation of expanded CAG repeats depends on hairpin formation (Zu et al., 2011), we sought to determine the structure of expanded GGGGCC repeats using the latest methodologies for generating secondary structure predictions (SSPs) (Gardner et al., 2011).”
RAN proteins and RNA foci from antisense transcripts in C9ORF72 ALS and frontotemporal dementia (2013, background): “Third, why are microsatellite expansion disorders typically adult onset diseases? Could aging or stress increase RAN protein expression or accumulation? Fourth, how do the repeat motifs and flanking sequences impact disease? Additionally, RAN translation across CAG expansions has been shown to occur”
C9orf72 repeat expansions cause neurodegeneration in Drosophila through arginine-rich proteins (2014, background): “The GGGGCC repeat can be translated in all sense and antisense frames, two of which encode the same DPR, resulting in five DPR proteins, all of which form inclusions in widespread brain regions (10, 12, 14-18).”
Targeting RNA foci in iPSC-derived motor neurons from ALS patients with C9ORF72 repeat expansion (2013, background): “Some noncoding repeats including those in DM1 and SCA8 were shown to produce non-ATG– initiated peptides, which could also play a role in disease pathogenesis (43); similarly generated peptides were recently demonstrated in C9-ALS patient tissue (16, 17).”
Liquid-Liquid Phase Separation in Disease. (2019, background): “Two principal types of phaseseparating molecules arise in repeat expansion disorders: (a) repeat RNAs and (b) repeat proteins that arise through repeat-associated noncanonical translation in all three reading frames (158, 159).”
RNA Phase Transitions in Repeat Expansion Disorders (2017, neutral): “RNAs can be non-canonically translated 10 to produce short peptides that can give rise to neurotoxicity 11 .”
Converging mechanisms in ALS and FTD: Disrupted RNA and protein homeostasis (2013, positive): “(Zu et al., 2011), leading to production, respectively, of potentially toxic RNA and protein species.”