Differences in biochemical abnormalities between knock-out aldh7a1 zebrafish and human PDE-ALDH7A1 are likely due to the early lethal phenotype in knock-out aldh7a1 zebrafish representing the most severe disease phenotype and decreased acetyl-CoA supply to TCA cycle due to lysine catabolism defect secondary to α-AASA dehydrogenase deficiency and decreased succinate supply due to glutamate decarboxylase and GABA transaminase dysfunction secondary to pyridoxal-5’-phosphate deficiency resulting in impaired energy production in human PDE-ALDH7A1. This evidence concerns the gene ALDH7A1 and hyperinsulinemic hypoglycemia, familial, 4.