Uroporphyrinogen III decarboxylase

  • OMIM: 176100
  • UniProt: P06132
  • MGI: 98 916

Uroporphyrinogencarboxylase ( UROD ) is the name of the enzyme that accomplishes the removal of four carboxyl groups from uroporphyrinogen III; This interaction is important for the biosynthesis of porphyrin in the metabolism of all living beings. Porphyrin is the man of the starting material for heme, vitamin B12, and other cofactors in plants for chlorophyll and also in the bacterial metabolism plays a role. A lack or a change in the UROD enzyme in certain places can cause in humans of a metabolic disease, porphyria cutanea tarda. Cause of the defect is always a mutation of the UROD gene.

Biosynthesis

The UROD gene is located on chromosome 1 in humans and its 10 exons spanning 3,430 base pairs. The transcript is 1300 bases in length and has, in the translational and post-translational modification, finally, the UROD enzyme with 367 amino acids.

Catalyzed reaction

4 CO2

Uroporphyrinogen III is four times decarboxylated to coproporphyrinogen III. It is noteworthy that the reaction does not require any cofactors.

Human Genome Organisation Mouse Genome Informatics Lyase Decarboxylation Organism
794670
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