| Fructose-1,6-bisphosphate aldolase (EC 4.1.2.13) is a tetrameric glycolytic enzyme that catalyzes the reversible conversion of fructose-1,6-bisphosphate to glyceraldehyde 3-phosphate and dihydroxyacetone phosphate. Vertebrates have 3 aldolase isozymes which are distinguished by their electrophoretic and catalytic properties. Differences indicate that aldolases A, B, and C are distinct proteins, the products of a family of related housekeeping genes exhibiting developmentally regulated expression of the different isozymes. The developing embryo produces aldolase A, which is produced in even greater amounts in adult muscle where it can be as much as 5% of total cellular protein. In adult liver, kidney and intestine, aldolase A expression is repressed and aldolase B is produced. In brain and other nervous tissue, aldolase A and C are expressed about equally. There is a highcatalytic activity:D-fructose 1,6-bisphosphate = glycerone phosphate + D-glyceraldehyde 3-phosphate.,disease:Defects in ALDOB are the cause of hereditary fructose intolerance (HFI) [MIM:229600]. HFI is an autosomal recessive disease that results in an inability to metabolize fructose and related sugars. Complete exclusion of fructose results in dramatic recovery, however, if not treated properly, HFI subjects suffer episodes of hypoglycemia, general ill condition, and risk of death the remainder of life.,miscellaneous:In vertebrates, three forms of this ubiquitous glycolytic enzyme are found, aldolase A in muscle, aldolase B in liver and aldolase C in brain.,pathway:Carbohydrate degradation, glycolysis, D-glyceraldehyde 3-phosphate and glycerone phosphate from D-glucose: step 4.,pathway:Carbohydrate degradation, glycolysis, D-glyceraldehyde 3-phosphate and glycerone phosphate from D-glucose: step 4/4.,similarity:Belongs to the class I fructose-bisphosphate aldolase family.,subunit:Homotetramer., |
