Some of them (Ftl1, Gstm1, Glyat, Sdha, Ces1d, Idh2 and Nipsnap1) can be corrected by relative iron depletion of Hjv−/− mice, while stress response proteins (Hnrnpdl, Ubxn1, Lap3 and Fabp1) appear to be suppressed by Hjv deficiency or DEN in an iron-independent manner. The gene discussed is IDH2; the disease is hyperinsulinemic hypoglycemia, familial, 4.