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Literature summary extracted from

  • Wang, Y.; Gatti, P.; Sadilek, M.; Scott, C.R.; Turecek, F.; Gelb, M.H.
    Direct assay of enzymes in heme biosynthesis for the detection of porphyrias by tandem mass spectrometry. Uroporphyrinogen decarboxylase and coproporphyrinogen III oxidase (2008), Anal. Chem., 80, 2599-2605.
    View publication on PubMedView publication on EuropePMC

Application

EC Number Application Comment Organism
1.3.3.3 analysis mass spectrometric assay for the two-step decarboxylative oxidation of coproporphyrinogen III to protoporphyrinogen IX catalyzed by CPO in mitochondria from human lymphocytes. The assay shows good reproducibility, uses simple workup by liquid-liquid extraction of enzymatic products, and employs commercially available substrates and internal standard. It was developed for use in clinical diagnostics of the inherited disorder hereditary coproporphyria Homo sapiens

KM Value [mM]

EC Number KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
1.3.3.3 0.000066
-
 Coproporphyrinogen-III pH 7.2, 37°C Homo sapiens
1.3.3.3 0.000066
-
 Coproporphyrinogen III in 250 mM Tris-HCl, 2.5 mM EDTA, pH 7.15, at 37°C Homo sapiens

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
1.3.3.3 mitochondrion
-
 Homo sapiens 5739
-
4.1.1.37 cytosol
-
 Homo sapiens 5829
-

Organism

EC Number Organism UniProt Comment Textmining
1.3.3.3 Homo sapiens
-
-
-
4.1.1.37 Homo sapiens
-
-
-

Source Tissue

EC Number Source Tissue Comment Organism Textmining
1.3.3.3 lymphocyte
-
 Homo sapiens
-
4.1.1.37 erythrocyte
-
 Homo sapiens
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
1.3.3.3 coproporphyrinogen-III + ?
-
 Homo sapiens protoporphyrinogen-IX + ?
-
 ?
1.3.3.3 coproporphyrinogen-III + O2 + 2 H+ CPO converts coproporphyrinogen III to protoporphyrinogen IX through harderoporphyrinogen IX Homo sapiens protoporphyrinogen-IX + 2 CO2 + 2 H2O
-
 ir
4.1.1.37 heptaporphyrinogen III formation of the stepwise decarboxylation products, hepta-, hexa-, penta-, and coproporphyrinogen III Homo sapiens hexaporphyrinogen III + CO2
-
 ?
4.1.1.37 hexaporphyrinogen III formation of the stepwise decarboxylation products, hepta-, hexa-, penta-, and coproporphyrinogen III Homo sapiens pentaporphyrinogen III + CO2
-
 ?
4.1.1.37 uroporphyrinogen I formation of the stepwise decarboxylation products, hepta-, hexa-, penta-, and coproporphyrinogen I Homo sapiens coproporphyrinogen I + 4 CO2
-
 ?
4.1.1.37 Uroporphyrinogen III
-
 Homo sapiens Coproporphyrinogen III + 4 CO2
-
 ?
4.1.1.37 uroporphyrinogen III formation of the stepwise decarboxylation products, hepta-, hexa-, penta-, and coproporphyrinogen III Homo sapiens heptaporphyrinogen III + CO2
-
 ?

Synonyms

EC Number Synonyms Comment Organism
1.3.3.3 coproporphyrinogen III oxidase
-
 Homo sapiens
1.3.3.3 CPO
-
 Homo sapiens
4.1.1.37 UroD
-
 Homo sapiens