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Direct assay of delta-aminolevulinic acid dehydratase in heme biosynthesis for the detection of porphyrias by tandem mass spectrometry

Choiniere, J.R.; Scott, C.R.; Gelb, M.H.; Turecek, F.; Anal. Chem. 82, 6730-6736 (2010)

Data extracted from this reference:

Application
EC Number
Application
Commentary
Organism
4.2.1.24
diagnostics
the enzyme activity is a diagnostic marker for the clinical diagnosis of delta-aminolevulinic acid dehydratase-deficient porphyria, a rare enzymatic deficiency of the heme biosynthetic pathway
Homo sapiens
Inhibitors
EC Number
Inhibitors
Commentary
Organism
Structure
4.2.1.24
Carbonate
using a carbonate buffer rather than phosphate causes nearly a 90% drop in activity in the developed assay method
Homo sapiens
4.2.1.24
Zn2+
exocenous addition of zinc results in a decrease by up to 35% in enzyme activity
Homo sapiens
KM Value [mM]
EC Number
KM Value [mM]
KM Value Maximum [mM]
Substrate
Commentary
Organism
Structure
4.2.1.24
0.333
-
5-aminolevulinate
pH 6.8, 37°C
Homo sapiens
Localization
EC Number
Localization
Commentary
Organism
GeneOntology No.
Textmining
4.2.1.24
cytosol
-
Homo sapiens
5829
-
Metals/Ions
EC Number
Metals/Ions
Commentary
Organism
Structure
4.2.1.24
Zn2+
required cofactor
Homo sapiens
Natural Substrates/ Products (Substrates)
EC Number
Natural Substrates
Organism
Commentary (Nat. Sub.)
Natural Products
Commentary (Nat. Pro.)
Organism (Nat. Pro.)
Reversibility
4.2.1.24
2 5-aminolevulinate
Homo sapiens
-
porphobilinogen + 2 H2O
-
-
?
Organism
EC Number
Organism
Primary Accession No. (UniProt)
Commentary
Textmining
4.2.1.24
Homo sapiens
-
-
-
Source Tissue
EC Number
Source Tissue
Commentary
Organism
Textmining
4.2.1.24
erythrocyte
-
Homo sapiens
-
Substrates and Products (Substrate)
EC Number
Substrates
Commentary Substrates
Literature (Substrates)
Organism
Products
Commentary (Products)
Literature (Products)
Organism (Products)
Reversibility
4.2.1.24
2 5-aminolevulinate
-
713748
Homo sapiens
porphobilinogen + 2 H2O
-
-
-
?
4.2.1.24
additional information
direct assay method development with incubation of erythrocyte lysate with the natural substrate, 5-aminolevulinate, followed by quantitative in situ conversion of porphobilinogen to its butyramide and mass spectrometric determination, overview. Using a carbonate buffer rather than phosphate causes nearly a 90% drop in activity and addition of zinc results in a further decrease by up to 35%
713748
Homo sapiens
?
-
-
-
-
Temperature Optimum [°C]
EC Number
Temperature Optimum [°C]
Temperature Optimum Maximum [°C]
Commentary
Organism
4.2.1.24
37
-
assay at
Homo sapiens
pH Optimum
EC Number
pH Optimum Minimum
pH Optimum Maximum
Commentary
Organism
4.2.1.24
6.8
-
assay at
Homo sapiens
Application (protein specific)
EC Number
Application
Commentary
Organism
4.2.1.24
diagnostics
the enzyme activity is a diagnostic marker for the clinical diagnosis of delta-aminolevulinic acid dehydratase-deficient porphyria, a rare enzymatic deficiency of the heme biosynthetic pathway
Homo sapiens
Inhibitors (protein specific)
EC Number
Inhibitors
Commentary
Organism
Structure
4.2.1.24
Carbonate
using a carbonate buffer rather than phosphate causes nearly a 90% drop in activity in the developed assay method
Homo sapiens
4.2.1.24
Zn2+
exocenous addition of zinc results in a decrease by up to 35% in enzyme activity
Homo sapiens
KM Value [mM] (protein specific)
EC Number
KM Value [mM]
KM Value Maximum [mM]
Substrate
Commentary
Organism
Structure
4.2.1.24
0.333
-
5-aminolevulinate
pH 6.8, 37°C
Homo sapiens
Localization (protein specific)
EC Number
Localization
Commentary
Organism
GeneOntology No.
Textmining
4.2.1.24
cytosol
-
Homo sapiens
5829
-
Metals/Ions (protein specific)
EC Number
Metals/Ions
Commentary
Organism
Structure
4.2.1.24
Zn2+
required cofactor
Homo sapiens
Natural Substrates/ Products (Substrates) (protein specific)
EC Number
Natural Substrates
Organism
Commentary (Nat. Sub.)
Natural Products
Commentary (Nat. Pro.)
Organism (Nat. Pro.)
Reversibility
4.2.1.24
2 5-aminolevulinate
Homo sapiens
-
porphobilinogen + 2 H2O
-
-
?
Source Tissue (protein specific)
EC Number
Source Tissue
Commentary
Organism
Textmining
4.2.1.24
erythrocyte
-
Homo sapiens
-
Substrates and Products (Substrate) (protein specific)
EC Number
Substrates
Commentary Substrates
Literature (Substrates)
Organism
Products
Commentary (Products)
Literature (Products)
Organism (Products)
Reversibility
4.2.1.24
2 5-aminolevulinate
-
713748
Homo sapiens
porphobilinogen + 2 H2O
-
-
-
?
4.2.1.24
additional information
direct assay method development with incubation of erythrocyte lysate with the natural substrate, 5-aminolevulinate, followed by quantitative in situ conversion of porphobilinogen to its butyramide and mass spectrometric determination, overview. Using a carbonate buffer rather than phosphate causes nearly a 90% drop in activity and addition of zinc results in a further decrease by up to 35%
713748
Homo sapiens
?
-
-
-
-
Temperature Optimum [°C] (protein specific)
EC Number
Temperature Optimum [°C]
Temperature Optimum Maximum [°C]
Commentary
Organism
4.2.1.24
37
-
assay at
Homo sapiens
pH Optimum (protein specific)
EC Number
pH Optimum Minimum
pH Optimum Maximum
Commentary
Organism
4.2.1.24
6.8
-
assay at
Homo sapiens
General Information
EC Number
General Information
Commentary
Organism
4.2.1.24
malfunction
delta-aminolevulinic acid dehydratase-deficient porphyria is a rare enzymatic deficiency of the heme biosynthetic pathway
Homo sapiens
4.2.1.24
metabolism
delta-aminolevulinic acid dehydratase is involved in the heme biosynthetic pathway
Homo sapiens
General Information (protein specific)
EC Number
General Information
Commentary
Organism
4.2.1.24
malfunction
delta-aminolevulinic acid dehydratase-deficient porphyria is a rare enzymatic deficiency of the heme biosynthetic pathway
Homo sapiens
4.2.1.24
metabolism
delta-aminolevulinic acid dehydratase is involved in the heme biosynthetic pathway
Homo sapiens