For instance, glutaric aciduria type II (GAII) has substantial overlap with Pptc7 KO presentation (hypoketotic hypoglycemia, increased circulating lactate, and elevation of multiple acylcarnitines)37, yet key distinctions remain (e.g., levels of α-aminoadipic acid, a lysine catabolite, are typically increased in GAII instead of decreased, as is seen in the Pptc7 KO)37. Here, PPTC7 is linked to multiple acyl-CoA dehydrogenase deficiency.