Literature summary for 4.1.2.13 extracted from

Malay, A.D.; Procious, S.L.; Tolan, D.R.
The temperature dependence of activity and structure for the most prevalent mutant aldolase B associated with hereditary fructose intolerance (2002), Arch. Biochem. Biophys., 408, 295-304.

Search for more literature for 4.1.2.13

Application

Application	Comment	Organism
medicine	mutations in aldolase B gene cause hereditary fructose intolerance, most common intolerance allele encodes a A149P substitution	Homo sapiens

Cloned(Commentary)

Cloned (Comment)	Organism
enzyme from patients with fructose intolerance	Homo sapiens

KM Value [mM]

KM Value [mM]	KM Value Maximum [mM]	Substrate	Comment	Organism	Structure
0.0017	-	D-fructose 1,6-bisphosphate	pH 7.4, 10°C, wild-type enzyme	Homo sapiens
0.0024	-	D-fructose 1,6-bisphosphate	pH 7.4, 30°C, wild-type enzyme	Homo sapiens
0.0028	-	D-fructose 1,6-bisphosphate	pH 7.4, 10°C, enzyme from patients with intolerance	Homo sapiens
0.0085	-	D-fructose 1,6-bisphosphate	pH 7.4, 30°C, enzyme from patients with intolerance	Homo sapiens
0.24	-	D-Fructose 1-phosphate	pH 7.4, 10°C, wild-type enzyme	Homo sapiens
0.88	-	D-Fructose 1-phosphate	pH 7.4, 30°C, wild-type enzyme	Homo sapiens
4.2	-	D-Fructose 1-phosphate	pH 7.4, 10°C, enzyme from patients with intolerance	Homo sapiens
16.4	-	D-Fructose 1-phosphate	pH 7.4, 30°C, enzyme from patients with intolerance	Homo sapiens

Molecular Weight [Da]

Molecular Weight [Da]	Molecular Weight Maximum [Da]	Comment	Organism
57000	-	sucrose density gradient centrifugation, 30°C	Homo sapiens
71000	-	sucrose density gradient centrifugation, 4°C	Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates	Organism	Comment (Nat. Sub.)	Natural Products	Comment (Nat. Pro.)	Rev.	Reac.
D-fructose 1,6-bisphosphate	Homo sapiens	-	glycerone phosphate + D-glyceraldehyde 3-phosphate	-	r

Organism

Organism	UniProt	Comment	Textmining
Homo sapiens	-	patients with fructose intolerance	-

Purification (Commentary)

Purification (Comment)	Organism
enzyme from patients with fructose intolerance	Homo sapiens

Substrates and Products (Substrate)

Substrates	Comment Substrates	Organism	Products	Comment (Products)	Rev.	Reac.
D-fructose 1,6-bisphosphate	-	Homo sapiens	glycerone phosphate + D-glyceraldehyde 3-phosphate	-	r
D-Fructose 1-phosphate	-	Homo sapiens	Glycerone phosphate + D-glyceraldehyde	-	?

Subunits

Subunits	Comment	Organism
dimer	gel filtration, enzyme from patients with fructose intolerance	Homo sapiens

Temperature Range [°C]

Temperature Minimum [°C]	Temperature Maximum [°C]	Comment	Organism
15	-	gradual loss of activity above, enzyme from patients with fructose intolerance	Homo sapiens
40	-	wild-type enzyme, activity is stable up to 40°C, declines sharply above 40°C	Homo sapiens

Temperature Stability [°C]

Temperature Stability Minimum [°C]	Temperature Stability Maximum [°C]	Comment	Organism
10	-	enzyme from patients with fructose intolerance shows increased temperature sensitivity, activity at 10°C is 6fold lower than that of the wild-type, and activity decreases substantially at higher temperatures	Homo sapiens

Turnover Number [1/s]

Turnover Number Minimum [1/s]	Turnover Number Maximum [1/s]	Substrate	Comment	Organism	Structure
0.008	-	D-Fructose 1-phosphate	pH 7.4, 30°C, enzyme from patients with intolerance	Homo sapiens
0.011	-	D-fructose 1,6-bisphosphate	pH 7.4, 30°C, enzyme from patients with intolerance	Homo sapiens
0.019	-	D-Fructose 1-phosphate	pH 7.4, 10°C, enzyme from patients with intolerance	Homo sapiens
0.029	-	D-fructose 1,6-bisphosphate	pH 7.4, 10°C, enzyme from patients with intolerance	Homo sapiens
0.12	-	D-Fructose 1-phosphate	pH 7.4, 10°C, wild-type enzyme	Homo sapiens
0.19	-	D-fructose 1,6-bisphosphate	pH 7.4, 10°C, wild-type enzyme	Homo sapiens
2	-	D-Fructose 1-phosphate	pH 7.4, 30°C, wild-type enzyme	Homo sapiens
2.1	-	D-fructose 1,6-bisphosphate	pH 7.4, 30°C, wild-type enzyme	Homo sapiens

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Release 2023.1 (January 2023)