Repeat-associated non-AUG translation from antisense CCG repeats in fragile X tremor/ataxia syndrome.

@article{Krans2016RepeatassociatedNT,
  title={Repeat-associated non-AUG translation from antisense CCG repeats in fragile X tremor/ataxia syndrome.},
  author={Amy Krans and Michael G. Kearse and Peter K. Todd},
  journal={Annals of neurology},
  year={2016},
  volume={80 6},
  pages={
          871-881
        }
}
OBJECTIVE Repeat-associated non-AUG (RAN) translation drives production of toxic proteins from pathogenic repeat sequences in multiple untreatable neurodegenerative disorders. Fragile X-associated tremor/ataxia syndrome (FXTAS) is one such condition, resulting from a CGG trinucleotide repeat expansion in the 5' leader sequence of the FMR1 gene. RAN proteins from the CGG repeat accumulate in ubiquitinated inclusions in FXTAS patient brains and elicit toxicity. In addition to the CGG repeat, an… CONTINUE READING
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