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Literature summary for 1.3.3.3 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

Application Comment Organism
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]

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

Localization

Localization Comment Organism GeneOntology No. Textmining
mitochondrion
-
Homo sapiens 5739
-

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
lymphocyte
-
Homo sapiens
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
coproporphyrinogen-III + ?
-
Homo sapiens protoporphyrinogen-IX + ?
-
?
coproporphyrinogen-III + O2 + 2 H+ CPO converts coproporphyrinogen III to protoporphyrinogen IX through harderoporphyrinogen IX Homo sapiens protoporphyrinogen-IX + 2 CO2 + 2 H2O
-
ir

Synonyms

Synonyms Comment Organism
coproporphyrinogen III oxidase
-
Homo sapiens
CPO
-
Homo sapiens