Interestingly, non-splice site nonsense mutations can cause exon-skipping through aberrant splicing [46], and accordingly, the c.553C > T mutation has been reported to cause partial transcriptional skipping of exon 6 of FANCC in an FA patient [45], a mechanism confirmed for HuH-7 in our study. Here, MIR7-3HG is linked to Friedreich ataxia.