Cloned (Comment) | Organism |
---|---|
expression of wild-type and mutants in Escherichia coli strain BL21 | Homo sapiens |
Protein Variants | Comment | Organism |
---|---|---|
D99G | site-directed mutagenesis, inactive holo-enzyme mutant existing as a complex with 2 substrate molecules covalently bound to the dipyrromethane cofactor | Homo sapiens |
additional information | the mutations of the enzyme cause acute intermittent porphyria | Homo sapiens |
R149Q | site-directed mutagenesis, inactive apo-enzyme which is unable to assemble with the dipyrromethane cofactor, the mutant enzyme is unstable and heat-labile | Homo sapiens |
R167Q | site-directed mutagenesis, mutation of active site residue, highly reduced activity compared to the wild-type enzyme, formation of stable enzyme-intermediate complexes | Homo sapiens |
R173Q | site-directed mutagenesis, highly reduced activity compared to the wild-type enzyme, the major part of the enzyme exists as apo-enzyme without assembled dipyrromethane cofactor | Homo sapiens |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
4 porphobilinogen + H2O | Homo sapiens | third reaction step in heme biosynthesis pathway | 1-hydroxymethylbilane + 4 NH3 | - |
? | |
additional information | Homo sapiens | mutations of the residues R167, R173, R149, and D99 of the enzyme cause acute intermittent porphyria, overview | ? | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Reaction | Comment | Organism | Reaction ID |
---|---|---|---|
4 porphobilinogen + H2O = hydroxymethylbilane + 4 NH3 | catalytic cycle, cofactor assembly and tetrapyrrole chain polymerization mechanism, C261 is involved, residues D99 and R167 are essential for activity, overview | Homo sapiens |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
4 porphobilinogen + H2O | third reaction step in heme biosynthesis pathway | Homo sapiens | 1-hydroxymethylbilane + 4 NH3 | - |
? | |
4 porphobilinogen + H2O | polymerization of 4 pyrrole molecules to a linear tetrapyrrole | Homo sapiens | hydroxymethylbilane + 4 NH3 | i.e. pre-uroporphyrinogen, highly unstable compound | ? | |
additional information | mutations of the residues R167, R173, R149, and D99 of the enzyme cause acute intermittent porphyria, overview | Homo sapiens | ? | - |
? |
Synonyms | Comment | Organism |
---|---|---|
More | formerly EC 4.3.1.8 | Homo sapiens |
porphobilinogen deaminase | - |
Homo sapiens |
Cofactor | Comment | Organism | Structure |
---|---|---|---|
dipyrromethane | cofactor assembly mechanism, R149 and R173 are important, cofactor is produced from the apo-enzyme with pre-uroporphyrinogen | Homo sapiens |