FAAP100 and Friedreich ataxia: Furthermore, lower levels of FAAP100WT are suggested in an FA-B line with the hemizygous pathogenic variant in FANCB c.832C>T predicting p.Q278* (25) compared with non-FA or FA-D2 cells (compare ratios on the corresponding blots below), suggesting destabilizing effects resulting from defects in the molecular interaction of FAAP100, FANCB, and FANCL.