SEC61A2, KHSRP, and SUGP2 were found to be significantly upregulated in FRDA, and KHSRP changed in the opposite direction in CT neurons vs. TT neurons, which might indicate that CT SIRT6 genotype results in a compensatory mechanism to “normalize” expression of this disrupted gene in FRDA. The gene discussed is SIRT6; the disease is Friedreich ataxia.