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Literature summary for 4.2.1.24 extracted from

  • Choiniere, J.R.; Scott, C.R.; Gelb, M.H.; Turecek, F.
    Direct assay of delta-aminolevulinic acid dehydratase in heme biosynthesis for the detection of porphyrias by tandem mass spectrometry (2010), Anal. Chem., 82, 6730-6736.
    View publication on PubMedView publication on EuropePMC
Search for more literature for 4.2.1.24

Application

Application Comment Organism
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

Inhibitors Comment Organism Structure
Carbonate using a carbonate buffer rather than phosphate causes nearly a 90% drop in activity in the developed assay method Homo sapiens
Zn2+ exocenous addition of zinc results in a decrease by up to 35% in enzyme activity Homo sapiens

KM Value [mM]

KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
0.333
-
 5-aminolevulinate pH 6.8, 37°C Homo sapiens

Localization

Localization Comment Organism GeneOntology No. Textmining
cytosol
-
 Homo sapiens 5829
-

Metals/Ions

Metals/Ions Comment Organism Structure
Zn2+ required cofactor Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
2 5-aminolevulinate Homo sapiens
-
 porphobilinogen + 2 H2O
-
 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
erythrocyte
-
 Homo sapiens
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
2 5-aminolevulinate
-
 Homo sapiens porphobilinogen + 2 H2O
-
 ?
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% Homo sapiens ?
-
 ?

Synonyms

Synonyms Comment Organism
ALAD
-
 Homo sapiens
delta-aminolevulinic acid dehydratase
-
 Homo sapiens

Temperature Optimum [°C]

Temperature Optimum [°C] Temperature Optimum Maximum [°C] Comment Organism
37
-
 assay at Homo sapiens

pH Optimum

pH Optimum Minimum pH Optimum Maximum Comment Organism
6.8
-
 assay at Homo sapiens

General Information

General Information Comment Organism
malfunction delta-aminolevulinic acid dehydratase-deficient porphyria is a rare enzymatic deficiency of the heme biosynthetic pathway Homo sapiens
metabolism delta-aminolevulinic acid dehydratase is involved in the heme biosynthetic pathway Homo sapiens