Cloned (Comment) | Organism |
---|---|
the enzyme is expressed into a housekeeping or an erythroid form as a result of differential promoter usage and splicing, three pairs of isoallelic forms, expression of two of the isoallelic forms, K210 and E210, with mutations involved in acute intermittent porphyria, AIP, in Escherichia coli as GST fusion proteins | Homo sapiens |
Protein Variants | Comment | Organism |
---|---|---|
additional information | mutations involved in acute intermittent porphyria, AIP, lead to an inactivation of the mutant enzyme, the mutations influence the structure and function of the enzyme | Homo sapiens |
KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|
additional information | - |
additional information | no differences in kinetics for the housekeeping and the erythroid enzyme form, overview | Homo sapiens | |
0.007 | - |
porphobilinogen | genetic variant isozyme E210 | Homo sapiens | |
0.009 | - |
porphobilinogen | genetic variant isozyme K210 | Homo sapiens |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
4 porphobilinogen + H2O | Homo sapiens | enzyme defects are involved in acute intermittent porphyria, AIP | hydroxymethylbilane + 4 NH3 | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Purification (Comment) | Organism |
---|---|
recombinant GST-tagged isoallelic forms K210 and E210 mutants from Escherichia coli to homogeneity | Homo sapiens |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
4 porphobilinogen + H2O | - |
Homo sapiens | hydroxymethylbilane + 4 NH3 | - |
? | |
4 porphobilinogen + H2O | enzyme defects are involved in acute intermittent porphyria, AIP | Homo sapiens | hydroxymethylbilane + 4 NH3 | - |
? |
Synonyms | Comment | Organism |
---|---|---|
PBG-D | - |
Homo sapiens |
porphobilinogen deaminase | - |
Homo sapiens |