The liver is the primary organ where fatty acid oxidation occurs, and children affected by MTP deficiency usually exhibit very early hepatic dysfunction.[37] To recapitulate the hepatic pathological phenotypes associated with pediatric FAO deficiency and the potential OXPHOS dysfunction, we fed 4‐month‐old HADHA‐HET mice with HFD for 2 months and then examined the liver for pathology and associated OXPHOS features. This evidence concerns the gene HADHA and hyperinsulinemic hypoglycemia, familial, 4.