Hereditary fructose intolerance (HFI) is a metabolic disease caused by the absence of an enzyme called aldolase B. In people with HFI, ingestion of fructose (fruit sugar) and sucrose (cane or …

2015年12月17日 · Hepatic formation of glycogen from fructose is principally catalyzed by aldolase B. HFI results from loss of aldolase B function. Normally aldolase B rapidly converts …

Hereditary fructose intolerance (HFI) is a pathological condition that occurs due to a deficiency of enzyme aldolase B. It is characterized by hypoglycemia, lactic acidosis, hypophosphatemia, …

Hereditary fructose intolerance (HFI) is an inborn error of fructose metabolism caused by a deficiency of the enzyme aldolase B. [1] Individuals affected with HFI are asymptomatic until …

2023年4月17日 · Fructose 1-phosphate aldolase deficiency or hereditary fructose intolerance (HFI) is an autosomal recessive disorder caused by the deficiency in aldolase B (fructose-1, 6 …

HFI results from a deficiency of aldolase B activity in the liver, kidney, and intestine (7). The disease is inherited as an autosomal recessive trait and most parents of HFI patients and …

HFI is a congenital error in fructose metabolism, an autosomal recessive metabolic disease resulting from a deficiency in aldolase B gene function [3]. First described in 1956, HFI is …

2023年5月29日 · Hereditary fructose intolerance is an autosomal recessive disease characterized by the absence of the enzyme aldolase B. Aldolase B is an essential enzyme that is …

Hereditary fructose intolerance (HFI) is a rare inborn disease characterized by a deficiency in aldolase B, which catalyzes the cleavage of fructose 1,6-bisphosphate and fructose 1 …

Hereditary fructose intolerance (HFI, OMIM 22960), caused by catalytic deficiency of aldolase B (fructose-1,6-bisphosphate aldolase, EC 4.1.2.13), is a recessively inherited condition in which …