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 | 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 Maximum [mM] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|
0.333 | - |
5-aminolevulinate | pH 6.8, 37°C | Homo sapiens |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
cytosol | - |
Homo sapiens | 5829 | - |
Metals/Ions | Comment | Organism | Structure |
---|---|---|---|
Zn2+ | required cofactor | Homo sapiens |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
2 5-aminolevulinate | Homo sapiens | - |
porphobilinogen + 2 H2O | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
erythrocyte | - |
Homo sapiens | - |
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 | Comment | Organism |
---|---|---|
ALAD | - |
Homo sapiens |
delta-aminolevulinic acid dehydratase | - |
Homo sapiens |
Temperature Optimum [°C] | Temperature Optimum Maximum [°C] | Comment | Organism |
---|---|---|---|
37 | - |
assay at | Homo sapiens |
pH Optimum Minimum | pH Optimum Maximum | Comment | Organism |
---|---|---|---|
6.8 | - |
assay at | Homo sapiens |
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 |