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- W3139096979 abstract "Repeat-associated non-AUG (RAN) translation was discovered in 2011 in spinocerebellar ataxia type 8 (SCA8) and myotonic dystrophy type 1 (DM1). This non-canonical form of translation occurs in all reading frames from both coding and non-coding regions of sense and antisense transcripts carrying expansions of trinucleotide to hexanucleotide repeat sequences. RAN translation has since been reported in 7 of the 53 known microsatellite expansion disorders which mainly present with neurodegenerative features. RAN translation leads to the biosynthesis of low-complexity polymeric repeat proteins with aggregating and cytotoxic properties. However, the molecular mechanisms and protein factors involved in assembling functional ribosomes in absence of canonical AUG start codons remain poorly characterised while secondary repeat RNA structures play key roles in initiating RAN translation. Here, we briefly review the repeat expansion disorders, their complex pathogenesis and the mechanisms of physiological translation initiation together with the known factors involved in RAN translation. Finally, we discuss research challenges surrounding the understanding of pathogenesis and future directions that may provide opportunities for the development of novel therapeutic approaches for this group of incurable neurodegenerative diseases." @default.
- W3139096979 created "2021-03-29" @default.
- W3139096979 creator A5012448532 @default.
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- W3139096979 creator A5064049854 @default.
- W3139096979 creator A5086311082 @default.
- W3139096979 creator A5086502478 @default.
- W3139096979 date "2021-03-17" @default.
- W3139096979 modified "2023-09-25" @default.
- W3139096979 title "Mechanisms of repeat-associated non-AUG translation in neurological microsatellite expansion disorders" @default.
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