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Exp Hematol 14(7):626-35, 1986

et al reported that the process of transcription in FRDA patients is terminated prematurely at the upstream of the expanded GAA repeats and this also contributes to the formation of a novel, truncated and stable RNA ( FXN-ett ) is subjected to alternative, non-productive splicing and does not participate in the synthesis of a functional frataxin ( FXN locus may lead to the inhibition of RNA polymerase activity, thereby significantly slowing the rate of transcription ( FXN locus