Aldolase B is one of three known aldolase isoenzymes, and is found in kidney and small adult intestine where it is associated with aldolases A or C. Aldolase B catalyzes the reversible cleavage of fructose 1-phosphate into dihydroxyacetone phosphate and glyceraldehyde. It is involved in both glycolytic and gluconeogenic pathways, where its quaternary structure is critical for maintaining its full catalytic function. Hereditary fructose intolerance (HFI), a potentially fatal human autosomal recessive disease of carbohydrate metabolism, results with Aldolase B deficiency. Partially active Aldolase B, however, has been reported in an HFI individuals, which supports the hypothesis that adequate gluconeogenesis/glycolysis is maintained in HFI patients by the presence of partially active enzymes (1-4).
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