In this regard, it was reported that ACAD-2 deficiency leads to the accumulation of various derivative organic acids, such as isovaleric acid, 3-hydroxyisovaleric acid, isovaleryl (C5)-carnitine, and isovaleryl glycine, and ACAD-11 deficiency results in the accumulation of the dicarboxylates glutaric acid, 3-hydroxy-glutaric acid, and glutaconic acid in human patients [44]. Here, ACAD11 is linked to hyperinsulinemic hypoglycemia, familial, 4.