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

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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