Biochemistry Carbohydrates db2fc715 In hereditary fructose intolerance there is defect in: A Phosphofructokinase B Fructose 2, 6-biphosphatase C Fructokinase D Aldolase B High-Yield Explanation Ans. D. Aldolase BDefect in hepatic fructokinase causes essential fructosuria.Defect in hepatic aldolase B causes hereditary fructose intolerance.