TNF-α-induced activation of NF-κB was higher in FANCD2-deficient cells (FA-D2, FA-D2/vec) than in FANCD2-proficient cells (FA-D2/D2); similarly, FANCC-deficient cells (FA-C) had higher levels of TNF-α-induced NF-κB activation than did the FANCC-proficient cells (FA-C/FANCC)(Fig 1A) We also showed that transiently expression of FANCD2WT repressed enhanced NF-κB transcriptional activity of FANCD2-deficient cells (FA-D2/vec). Here, NFKB1 is linked to Friedreich ataxia.