EC Number |
Natural Substrates |
---|
2.5.1.61 | 4 porphobilinogen + H2O |
third reaction step in heme biosynthesis pathway |
2.5.1.61 | 4 porphobilinogen + H2O |
- |
2.5.1.61 | 4 porphobilinogen + H2O |
enzyme defects are involved in acute intermittent porphyria, AIP |
2.5.1.61 | 4 porphobilinogen + H2O |
enzyme defects are involved in acute intermittent porphyria, AIP, a neuropathic disease |
2.5.1.61 | 4 porphobilinogen + H2O |
the enzyme is important in heme biosynthesis, a nonsense mutation in the porphobilinogen deaminase gene causes acute intermittent porphyria, AIP, overview, eight enzymes are involved in the heme synthesis and defects in seven of them cause porphyria, overview |
2.5.1.61 | 4 porphobilinogen + H2O |
the enzyme is important in heme biosynthesis, a nonsense mutation in the porphobilinogen deaminase gene causes chester porphyria, existence of dual porphyrias with two enzymes of heme biosynthesis being deficient simultaneously, overview |
2.5.1.61 | 4 porphobilinogen + H2O |
third step in the heme biosynthetic pathway, enzyme defects are involved in acute intermittent porphyria, AIP, an autosomal dominant disorder characterised by acute, potentially life-threatening neurological attacks that are precipitated by various drugs, reproductive hormones and other factors |
2.5.1.61 | more |
third enzyme in the heme biosynthetic pathway |
2.5.1.61 | more |
occurrence of multiple forms of the enzyme. These isomers correspond to the enzyme-substrate intermediates: mono-, di-, tri-, and tetrapyrrole |
2.5.1.61 | more |
in presence of a second enzyme, EC 4.2.1.75, uroporphyrinogen-III synthase, often called co-synthase, the product is cyclized to form uroporphyrinogen-III |