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Literature summary 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.
    View publication on PubMed

Application

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

Cloned(Commentary)

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

KM Value [mM]

EC Number KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
4.1.2.13 0.0017
-
D-fructose 1,6-bisphosphate pH 7.4, 10°C, wild-type enzyme Homo sapiens
4.1.2.13 0.0024
-
D-fructose 1,6-bisphosphate pH 7.4, 30°C, wild-type enzyme Homo sapiens
4.1.2.13 0.0028
-
D-fructose 1,6-bisphosphate pH 7.4, 10°C, enzyme from patients with intolerance Homo sapiens
4.1.2.13 0.0085
-
D-fructose 1,6-bisphosphate pH 7.4, 30°C, enzyme from patients with intolerance Homo sapiens
4.1.2.13 0.24
-
D-Fructose 1-phosphate pH 7.4, 10°C, wild-type enzyme Homo sapiens
4.1.2.13 0.88
-
D-Fructose 1-phosphate pH 7.4, 30°C, wild-type enzyme Homo sapiens
4.1.2.13 4.2
-
D-Fructose 1-phosphate pH 7.4, 10°C, enzyme from patients with intolerance Homo sapiens
4.1.2.13 16.4
-
D-Fructose 1-phosphate pH 7.4, 30°C, enzyme from patients with intolerance Homo sapiens

Molecular Weight [Da]

EC Number Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
4.1.2.13 57000
-
sucrose density gradient centrifugation, 30°C Homo sapiens
4.1.2.13 71000
-
sucrose density gradient centrifugation, 4°C Homo sapiens

Natural Substrates/ Products (Substrates)

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

Organism

EC Number Organism UniProt Comment Textmining
4.1.2.13 Homo sapiens
-
patients with fructose intolerance
-

Purification (Commentary)

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

Substrates and Products (Substrate)

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

Subunits

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

Temperature Range [°C]

EC Number Temperature Minimum [°C] Temperature Maximum [°C] Comment Organism
4.1.2.13 15
-
gradual loss of activity above, enzyme from patients with fructose intolerance Homo sapiens
4.1.2.13 40
-
wild-type enzyme, activity is stable up to 40°C, declines sharply above 40°C Homo sapiens

Temperature Stability [°C]

EC Number Temperature Stability Minimum [°C] Temperature Stability Maximum [°C] Comment Organism
4.1.2.13 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]

EC Number Turnover Number Minimum [1/s] Turnover Number Maximum [1/s] Substrate Comment Organism Structure
4.1.2.13 0.008
-
D-Fructose 1-phosphate pH 7.4, 30°C, enzyme from patients with intolerance Homo sapiens
4.1.2.13 0.011
-
D-fructose 1,6-bisphosphate pH 7.4, 30°C, enzyme from patients with intolerance Homo sapiens
4.1.2.13 0.019
-
D-Fructose 1-phosphate pH 7.4, 10°C, enzyme from patients with intolerance Homo sapiens
4.1.2.13 0.029
-
D-fructose 1,6-bisphosphate pH 7.4, 10°C, enzyme from patients with intolerance Homo sapiens
4.1.2.13 0.12
-
D-Fructose 1-phosphate pH 7.4, 10°C, wild-type enzyme Homo sapiens
4.1.2.13 0.19
-
D-fructose 1,6-bisphosphate pH 7.4, 10°C, wild-type enzyme Homo sapiens
4.1.2.13 2
-
D-Fructose 1-phosphate pH 7.4, 30°C, wild-type enzyme Homo sapiens
4.1.2.13 2.1
-
D-fructose 1,6-bisphosphate pH 7.4, 30°C, wild-type enzyme Homo sapiens