Furthermore, as the Toll-like receptor 4 (TLR4) is regulated by GLRX1 (Chantzoura et al., 2010; Moghimpour Bijani et al., 2012) and we recently found, by comparative transcriptomic analysis, a significant increase of TLR4 in one of two sisters both displaying fxn deficiency but only one of them symptomatic (Petrillo et al., 2024), we also measured the TLR4 content in FRDA fibroblasts, to investigate its possible involvement in the disease. The gene discussed is TLR4; the disease is hyperinsulinemic hypoglycemia, familial, 4.