Human variants of Coproporphyrinogen oxidase are cofactor-independent.
2.
Coproporphyrinogen III is the most common variance.
3.
This enzyme is responsible for catalyzing the conversion of uroporphyrinogen to coproporphyrinogen through the removal of four carboxymethyl side chains.
4.
CPOX, the sixth enzyme of the haem biosynthetic pathway, converts coproporphyrinogen III to protoporphyrinogen IX through two sequential steps of oxidative decarboxylation.
5.
Enzyme tests show markedly reduced activity of coproporphyrinogen oxidase, compared to both unaffected individuals and those affected with hereditary coproporphyria, consistent with recessive inheritance.
6.
The systematic name of this enzyme class is "'coproporphyrinogen-III : S-adenosyl-L-methionine oxidoreductase ( decarboxylating ) " '.
7.
In the metabolism of porphyrin, it is formed from uroporphyrinogen III by the enzyme uroporphyrinogen III decarboxylase, and it is converted into protoporphyrinogen IX by coproporphyrinogen III oxidase.
8.
:coproporphyrinogen III + 2 S-adenosyl-L-methionine \ rightleftharpoons protoporphyrinogen IX + 2 CO 2 + 2 L-methionine + 2 5'- deoxyadenosine
9.
Uroporphyrinogen III decarboxylase ( UroD ) is a homodimeric enzyme (, ) that catalyzes the fifth step in heme biosynthesis : the elimination of carboxyl groups from the four acetate side chains of uroporphyrinogen III to yield coproporphyrinogen III.
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