Finally, a multi-omic (metabolomic and transcriptomic) analysis of cardiac mitochondrial stress in three mouse models with different degrees of frataxin deficiency (YG8-800, KIKO-700, and FXNG127V) showed an increase in metabolites related to glutathione and purine metabolism, the citric acid cycle (tricarboxylic acid cycle), and several amino acid-related metabolic pathways in female KIKO-700 hearts, as well as changes in only six metabolites (4-pyridoxic acid, NADH, kynurenic acid, glutathione, deoxyribose 5-phosphate, and guanosine triphosphate) in YG8-800 hearts. Here, FXN is linked to hyperinsulinemic hypoglycemia, familial, 4.