What is aldolase B deficiency?
Aldolase B is the key enzyme in fructose metabolism, and its deficiency can lead to a toxic accumulation of fructose-1-phosphate. Fructose is rapidly converted into fructose-1-phosphate by fructokinase, leading to depletion of inorganic phosphate and ATP.
What is aldolase deficiency?
Aldolase A deficiency, is an autosomal recessive metabolic disorder resulting in a deficiency of the enzyme aldolase A; the enzyme is found predominantly in red blood cells and muscle tissue.
Why does aldolase B cause hypoglycemia?
Hypoglycemia in the toddler and child Aldolase splits fructose 1-phosphate to dihydroxy-acetone phosphate and glyceraldehyde, substrates for gluconeogenesis via fructose 1,6-diphosphate. Fructose 1-phosphate accumulates in liver and kidney cells causing phosphate depletion and gradual long-term organ damage.
What does aldolase B do?
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.
Is aldolase B reversible?
The generic fructose 1,6-bisphosphate aldolase enzyme catalyzes the reversible cleavage of fructose 1,6-bisphosphate (FBP) into glyceraldehyde 3-phosphate and dihydroxyacetone phosphate (DHAP) as well as the reversible cleavage of fructose 1-phosphate (F1P) into glyceraldehyde and dihydroxyacetone phosphate.
Is fructan intolerance genetic?
Hereditary fructose intolerance is a genetic disorder. Your body lacks the necessary protein or enzyme that’s needed to break down fructose when you have fructose intolerance. That means your body can’t digest the fructose found in many different foods.
What enzyme class is aldolase?
class I protein
The aldolase used in gluconeogenesis and glycolysis is a cytoplasmic protein. Three forms of class I protein are found in vertebrates. Aldolase A is preferentially expressed in muscle and brain; aldolase B in liver, kidney, and in enterocytes; and aldolase C in brain.
What does aldolase do in gluconeogenesis?
Aldolase catalyzes the cleavage of this hexose into two triose phosphates, G3P and DHAP. The reverse reaction, synthesis of Fru 1,6-P2 from these triose phosphates in the gluconeogenic pathway, is also catalyzed by aldolase.
Where is aldolase produced?
This enzyme is most likely found in all cells. But,more significant concentrations are commonly found in muscle, brain, and liver tissues. People with increased levels of aldolase were found to have a myotonic muscle disease. Aldolase is found in the cytoplasm and nucleus of cells.
Why is aldolase named aldolase?
Glycolysis, a catabolic pathway, uses the forward reaction. Aldolase is divided into two classes by mechanism. The word aldolase also refers, more generally, to an enzyme that performs an aldol reaction (creating an aldol) or its reverse (cleaving an aldol), such as Sialic acid aldolase, which forms sialic acid.
Where is aldolase A found?