RAN Translation of the Expanded CAG Repeats in the SCA3 Disease Context

Magdalena Jazurek-Ciesiolka,Adam Ciesiolka,Alicja A. Komur,Martyna O. Urbanek-Trzeciak,Agnieszka Fiszer

Journal of Molecular Biology（2020）

引用 16|浏览16

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摘要

•Expanded CAG repeats in the ATXN3 sequence context support RAN translation.•SCA3 RAN polyQ and polyA proteins are toxic to cells.•ATXN3 flanking sequences strongly influence RAN translation initiation and efficiency.•SCA3 RAN polyQ proteins are initiated at non-cognate codons upstream of CAG repeats.•SCA3 RAN translation is promoted by integrated stress response.

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关键词

ATXN3,c9ALS/FTD,CHOP,DM1,ER,FECD,FXTAS,iMet-tRNA,ISR,MetAPs,NAT,NPC,polyA,polyQ,polyS,RAN translation,SA,SCA3,TG

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