ASAH1 and hyperinsulinemic hypoglycemia, familial, 4: Animal models of ACDase deficiency show significant damage to the liver and change to lipid profiles and metabolism, including hepatomegaly with higher serum levels of aspartate, aminotransferase, alanine aminotransferase, and alkaline phosphatase and decreased levels of free fatty acids, triglycerides, and cholesterol [25].