Why aldolase deficiency is more severe in fructose metabolism?

In individuals deficient in aldolase B activity, this causes an accumulation of fructose-1-phosphate, leading to subsequent inhibition of both glycolytic and gluconeogenesis pathways, thus causing hypoglycemia in individuals.

What does fructose-1-phosphate aldolase do?

h. Hereditary fructose intolerance is the result of a deficiency of the enzyme fructose-1-phosphate aldolase, which causes fructose-1-phosphate to accumulate in the liver. Fructose-1-phosphate is a competitive inhibitor of phosphorylase, an enzyme that regulates the conversion of glycogen to glucose.

What causes aldolase B deficiency?

Fructose Intolerance and Essential Fructosuria Fructose intolerance is an autosomal recessive disease, caused by a genetic defect in fructose 1-phosphate aldolase (aldolase B) in the liver. The symptoms of aldolase B deficiency start when the infant is exposed to fructose.

Which enzyme is deficient in fructose intolerance?

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 beet sugar, table sugar) causes severe hypoglycemia (low blood sugar) and the build up of dangerous substances in the liver.

How is aldolase B involved in the metabolism of fructose?

Aldolase B is involved in the breakdown (metabolism) of the simple sugar fructose, which is found mostly in fruits and is used in the body for energy. Aldolase B is responsible for the second step in the metabolism of fructose, which breaks down the molecule fructose-1-phosphate into glyceraldehyde and dihydroxyacetone phosphate.

Where is aldolase B found in the body?

Aldolase B is found primarily in the liver, but it is also present at lower levels in kidney and intestinal cells. Aldolase B is involved in the breakdown (metabolism) of the simple sugar fructose, which is found mostly in fruits and is used in the body for energy.

How does a deficiency in aldolase B cause liver damage?

A deficiency in the enzyme Aldolase B may cause the accumulation of the fructose 1-phosphate in the liver cell, small intestine and kidney and thus make our body unable to convert the fructose into simpler sugars and as a result the sugar level of the body will fall and cause the formation of the toxic substance that damage our liver.

How are mutations in ALDOB related to fructose intolerance?

ALDOB gene mutations reduce the function of the enzyme, impairing its ability to metabolize fructose. A lack of functional aldolase B results in an accumulation of fructose-1-phosphate in liver cells.