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A mistranslation-prone transcriptome underlying polyglutamine expansion diseases

We propose a mechanism underlying polyglutamine (polyQ) repeat expansion diseases, in which the translation of expanded CAG codon repeats leads to translation stress. The stress is generated by depletion of the cognate glutaminyl-tRNA pool, which could cause ribosome frameshifting and lead to mistranslation of transcripts with specific characteristics.

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Correspondence to Michele Vendruscolo.

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The authors declare no competing interests.

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Buhr, F., Ciryam, P.S. & Vendruscolo, M. A mistranslation-prone transcriptome underlying polyglutamine expansion diseases. Nat Rev Mol Cell Biol 22, 583–584 (2021).

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