FIG. 6.

Progressive expansion of the GAA repeats in FRDA iPSCs correlates with decreased expression of FXN mRNA. (A, C, E) Analyses of the GAA repeat size in FRDA iPSCs subcultured for up to 24 passages (p). Genomic DNA from the iPSC cultures was isolated at the indicated passage numbers and the repeat length was determined using long GAA PCR as described in Materials and Methods. (B, D, E) In parallel, at the indicated passages, total RNA was extracted to measure FXN mRNA levels. Determination of GAA repeat expansion and FXN mRNA expression in FRDA iPSCs obtained in the presence of DMSO (A and B, respectively), NaB (C, D, respectively), and Parnate (E, F, respectively). *Indicates P < 0.05.