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 [transition]

Species

Homo sapiens

Compartment

cytosol

Synonyms

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

ReviewStatus

5/5

Locations in the PathwayBrowser

Expand all

General

SBML | | PDF

SVG | | PPTX | SBGN

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

Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

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