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Although expression studies showed that both the compound heterozygous carriers for the expanded GAA and the (GAAGGA) 66 – 67 repeat showed a significant reduction of FXN mRNA and protein levels in their leukocytes and fibroblasts (Santoro et al., 2020), none of them developed any disease manifestation, supporting that this array represents a benign variant as previously proposed by Ohshima et al
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