ⓘ Uroporphyrinogen III synthase

                                     

ⓘ Uroporphyrinogen III synthase

Uroporphyrinogen III synthase EC 4.2.1.75 is an enzyme involved in the metabolism of the cyclic tetrapyrrole compound porphyrin. It is involved in the conversion of hydroxymethyl bilane into uroporphyrinogen III. This enzyme catalyses the inversion of the final pyrrole unit of the linear tetrapyrrole molecule, linking it to the first pyrrole unit, thereby generating a large macrocyclic structure, uroporphyrinogen III. The enzyme folds into two alpha/beta domains connected by a beta-ladder, the active site being located between the two domains.

                                     
  • hydroxymethylbilane by the enzyme uroporphyrinogen III synthase and is further converted into coproporphyrinogen III by the enzyme uroporphyrinogen III decarboxylase
  • Uroporphyrinogen III is a tetrapyrrole, the first macrocyclic intermediate in the biosynthesis of heme, chlorophyll, vitamin B12, and siroheme. It is a
  • mutation it causes a uroporphyrinogen III synthase and uroporphyrinogen cosynthase defect. When the enzyme uroporphyrinogen III synthase is reacting normally
  • porphobilinogen by the enzyme porphobilinogen deaminase: The enzyme uroporphyrinogen III synthase closes the chain to form a porphyrinogen a class of compounds
  • iron - containing porphinoid sirohaem. CysG synthesizes sirohaem from uroporphyrinogen III via reactions which encompass two branchpoint intermediates in tetrapyrrole
  • coproporphyrinogen III as the primary product. This is accomplished by the clockwise removal of the four carboxyl groups present in the cyclic uroporphyrinogen III molecule
  • hydroxymethylbilane synthase PPOX: protoporphyrinogen oxidase UROD: uroporphyrinogen decarboxylase UROS: uroporphyrinogen III synthase congenital erythropoietic
  • variegate porphyria UROD: uroporphyrinogen decarboxylase deficiency causes porphyria cutanea tarda UROS: uroporphyrinogen III synthase deficiency causes congenital
  • cyclizing EC 4.2.1.74: long - chain - enoyl - CoA hydratase EC 4.2.1.75: uroporphyrinogen - III synthase EC 4.2.1.76: UDP - glucose 4, 6 - dehydratase EC 4.2.1.77: trans - L - 3 - hydroxyproline
  • erythropoietic protoporphyria associated with ALAS2 aminolevulinic acid synthase has also been described. X - linked dominant erythropoietic protoporphyria