KL and Friedreich ataxia: Increased renal levels and activation of NF-κB in FA-AKI mice was apparent from higher cytoplasmic as well as nuclear p65 levels, respectively (Fig. 9A,B) (Supplementary Fig. S5), increased Lcn2 (Fig. 9C) and Fn14 (Fig. 9D) mRNA levels, and decreased mRNA expression of the negatively regulated NF-κB target, Klotho (Fig. 9E).