Coproporphyrinogen III
Encyclopedia
In the metabolism of porphyrin
, the enzyme uroporphyrinogen III decarboxylase
generates coproporphyrinogen III from uroporphyrinogen III
, and coproporphyrinogen III oxidase
converts it into protoporphyrinogen IX
.
Porphyrin
Porphyrins are a group of organic compounds, many naturally occurring. One of the best-known porphyrins is heme, the pigment in red blood cells; heme is a cofactor of the protein hemoglobin. Porphyrins are heterocyclic macrocycles composed of four modified pyrrole subunits interconnected at...
, the enzyme uroporphyrinogen III decarboxylase
Uroporphyrinogen III decarboxylase
Uroporphyrinogen decarboxylase, also known as UROD, is an enzyme that in humans is encoded by the UROD gene.- Function :This gene encodes the fifth enzyme of the heme biosynthetic pathway...
generates coproporphyrinogen III from uroporphyrinogen III
Uroporphyrinogen III
Uroporphyrinogen III is an metabolic intermediate in the biosynthesis of protoporphyrin. It is created by the enzyme uroporphyrinogen III synthase, and is converted into coproporphyrinogen III by the enzyme uroporphyrinogen III decarboxylase.-See also:...
, and coproporphyrinogen III oxidase
Coproporphyrinogen III oxidase
Coproporphyrinogen-III oxidase, mitochondrial is an enzyme that in humans is encoded by the CPOX gene. A genetic defect in the enzyme results in a reduced production of heme in animals. The medical condition associated with this enzyme defect is called hereditary coproporphyria...
converts it into protoporphyrinogen IX
Protoporphyrinogen IX
Protoporphyrinogen IX is a precursor for protoporphyrin IX. See Porphyrins for the pathway and more information.-See also:* Protoporphyrinogen oxidase...
.