EC Number   |
Natural Substrates |
|---|
    4.2.1.75 | Hydroxymethylbilane |
- |
| 4.2.1.75 | hydroxymethylbilane |
linear tetrapyrrole, fourth step in the biosynthesis of porphyrin, essential reaction, decreased enzyme activity leads to the autosomal recessive disorder congenital erythropetic porphyria |
| 4.2.1.75 | hydroxymethylbilane |
macrocyclic, role of the enzyme in tetrapyrrole based copound biosynthesis, overview |
| 4.2.1.75 | hydroxymethylbilane |
fourth enzyme in heme biosynthesis. Congenital erythropoietic porphyria is a very rare disease that is inherited as an autosomal recessive trait and results from a profound deficiency of uroporphyrinogen III cosynthase, the fourth enzyme in heme biosynthesis. The degree of severity of clinical symptoms mainly depends on the amount of residual uroporphyrinogen III cosynthase activity |
| 4.2.1.75 | Hydroxymethylbilane |
synthesis of uroporphyrinogen III, key intermediate for biosynthesis of tetrapyrrolic compounds like chlorophylls, hemes, cytochromes and vitamin B12 |
| 4.2.1.75 | hydroxymethylbilane |
a step in tetrapyrrole biosynthesis, e.g. of chlorophyll, haem, sirohaem and bilins, overview |
| 4.2.1.75 | hydroxymethylbilane |
intramolecular rearrangement of the d-pyrrole group and ring closure |
| 4.2.1.75 | hydroxymethylbilane |
the D-ring of the hydroxymethylbilane substrate binds to the enzyme in a conformation that shields its terminal portion from reacting with ring A and prevents the formation of the biologically useless uroporphyrinogen I, reaction mechanism, overview |
| 4.2.1.75 | hydroxymethylbilane |
the enzyme catalyzes the cyclization and D-ring isomerization of hydroxymethylbilane to uroporphyrinogen III, the cyclic tetrapyrrole and physiologic precursor of heme, chlorophyl, and corrin |
| 4.2.1.75 | more |
uroporphyrinogen III synthase functions as heme synthesis enzyme during hematopoietic development of Danio rerio |
