FIG. 4.

Effect on protein expression of adding BNA-1 (12.5 nM) to homozygous FA patient-derived cell lines or a cell line derived from a heterozygous donor. (a) Sequences of BNA-1 and BNA-2. Data from parallel experiments using BNA-2 are shown in Supplementary Figure S3. (b) Western analysis of FXN protein expression in different FRDA cell lines after addition of BNA-1. (c) Quantification of data shown in (b), n = 3. Numbers of GAA repeats in two alleles of patient FXN genes are shown in parentheses.