Reactome | ALDOB tetramer cleaves Fru-1-P to GA and DHAP

ALDOB tetramer cleaves Fru-1-P to GA and DHAP

Stable Identifier

R-HSA-70342

Type

Reaction

Species

Homo sapiens

Compartment

cytosol

Synonyms

Aldolase B (ALDOB) cleaves fructose-1-phosphate to form D-glyceraldehyde and dihydroxyacetone phosphate, D-fructose-1-phosphate <=> D-glyceraldehyde + dihydroxyacetone phosphate

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ALDOB tetramer cleaves Fru-1-P to GA and DHAP

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Cytosolic aldolase B (ALDOB) catalyzes the reaction of D-fructose 1-phosphate (Fru 1-P) to form dihydroxyacetone phosphate (DHAP) and D-glyceraldehyde (GA) (Hers & Kusaka 1953; Schapira 1975). The active form of the enzyme is a tetramer (Dalby et al. 2001). Deficiencies in the enzyme are associated with hereditary fructose intolerance in vivo (e.g., Tolan 1995; Ali et al. 1998).
ALDOB is the same aldolase isoform that catalyzes the reversible cleavage of fructose-1,6-bisphosphate in glycolysis. This isoform, found in liver, kidney, and intestine, is approximately equally active with fructose 1 phosphate and fructose 1,6 bisphosphate as substrates at saturating concentrations, while the muscle and brain isoforms (ALDOA and ALDOC, respectively), have little activity with fructose-1-phosphate (Lebherz & Rutter 1969; Penhoet et el. 1969).

Literature References

PubMed ID	Title	Journal	Year
8535439	Molecular basis of hereditary fructose intolerance: mutations and polymorphisms in the human aldolase B gene Tolan, DR	Hum Mutat	1995
5353106	Isolation of fructose diphosphate aldolases A, B, and C Kochman, M, Rutter, WJ, Penhoet, EE	Biochemistry	1969
11679716	The structure of human liver fructose-1,6-bisphosphate aldolase Littlechild, JA, Tolan, DR, Dalby, AR	Acta Crystallogr D Biol Crystallogr	2001
13093749	[The metabolism of fructose-1-phosphate in the liver.] Hers, HG, Kusaka, T	Biochim Biophys Acta	1953
5777313	Distribution of fructose diphosphate aldolase variants in biological systems Rutter, WJ, Lebherz, HG	Biochemistry	1969
805723	Kinetic and immunological abnormalities of aldolase B in hereditary fructose intolerance Schapira, F	Biochem. Soc. Trans.	1975
9610797	Hereditary fructose intolerance Cox, TM, Ali, M, Rellos, P	J. Med. Genet.	1998