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Literature summary extracted from

  • Morgan, R.R.; da, S.V.; Puy, H.; Deybach, J.C.; Elder, G.H.
    Functional studies of mutations in the human protoporphyrinogen oxidase gene in variegate porphyria (2002), Cell. Mol. Biol., 48, 79-82.
    View publication on PubMed

Cloned(Commentary)

EC Number Cloned (Comment) Organism
1.3.3.4 expression of wild-type and mutant enzymes in Escherichia coli enzyme-deficient strain SAS38X which is not able to grow without exogenously added heme, complementation study, comparison of missense mutations in the human PPOX gene with the equivalent positions of other species' genes Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
1.3.3.4 A172V site-directed mutagenesis, 99.8% reduced activity compared to the wild-type enzyme, 99% complementation of enzyme-deficient Escherichia coli strain SAS38X Homo sapiens
1.3.3.4 D143V site-directed mutagenesis, 99.6% reduced activity compared to the wild-type enzyme, 89% complementation of enzyme-deficient Escherichia coli strain SAS38X Homo sapiens
1.3.3.4 del281H site-directed mutagenesis, inactive mutant, no complementation of enzyme-deficient Escherichia coli strain SAS38X Homo sapiens
1.3.3.4 G232R site-directed mutagenesis, inactive mutant, no complementation of enzyme-deficient Escherichia coli strain SAS38X Homo sapiens
1.3.3.4 G40E site-directed mutagenesis, inactive mutant, no complementation of enzyme-deficient Escherichia coli strain SAS38X Homo sapiens
1.3.3.4 G453R site-directed mutagenesis, nearly inactive mutant, but 89% complementation of enzyme-deficient Escherichia coli strain SAS38X Homo sapiens
1.3.3.4 G453V site-directed mutagenesis, inactive mutant, no complementation of enzyme-deficient Escherichia coli strain SAS38X Homo sapiens
1.3.3.4 L154P site-directed mutagenesis, 99.6% reduced activity compared to the wild-type enzyme, 93% complementation of enzyme-deficient Escherichia coli strain SAS38X Homo sapiens
1.3.3.4 L15F site-directed mutagenesis, 97.4% reduced activity compared to the wild-type enzyme, 49% complementation of enzyme-deficient Escherichia coli strain SAS38X Homo sapiens
1.3.3.4 L295P site-directed mutagenesis, inactive mutant, no complementation of enzyme-deficient Escherichia coli strain SAS38X Homo sapiens
1.3.3.4 L444P site-directed mutagenesis, inactive mutant, no complementation of enzyme-deficient Escherichia coli strain SAS38X Homo sapiens
1.3.3.4 L73P site-directed mutagenesis, 97.6% reduced activity compared to the wild-type enzyme, complete complementation of enzyme-deficient Escherichia coli strain SAS38X Homo sapiens
1.3.3.4 L85P site-directed mutagenesis, inactive mutant, no complementation of enzyme-deficient Escherichia coli strain SAS38X Homo sapiens
1.3.3.4 R152C site-directed mutagenesis, 99.4% reduced activity compared to the wild-type enzyme, 86% complementation of enzyme-deficient Escherichia coli strain SAS38X Homo sapiens
1.3.3.4 R168H site-directed mutagenesis, 99.8% reduced activity compared to the wild-type enzyme, 68% complementation of enzyme-deficient Escherichia coli strain SAS38X Homo sapiens
1.3.3.4 R38P site-directed mutagenesis, 99.2% reduced activity compared to the wild-type enzyme, 59% complementation of enzyme-deficient Escherichia coli strain SAS38X Homo sapiens
1.3.3.4 S350P site-directed mutagenesis, inactive mutant, no complementation of enzyme-deficient Escherichia coli strain SAS38X Homo sapiens
1.3.3.4 V158M site-directed mutagenesis, 91.8% reduced activity compared to the wild-type enzyme, 97% complementation of enzyme-deficient Escherichia coli strain SAS38X Homo sapiens
1.3.3.4 V282D site-directed mutagenesis, inactive mutant, no complementation of enzyme-deficient Escherichia coli strain SAS38X Homo sapiens
1.3.3.4 V290L site-directed mutagenesis, 98.8% reduced activity compared to the wild-type enzyme, 93% complementation of enzyme-deficient Escherichia coli strain SAS38X Homo sapiens
1.3.3.4 V335G site-directed mutagenesis, inactive mutant, no complementation of enzyme-deficient Escherichia coli strain SAS38X Homo sapiens
1.3.3.4 V84G site-directed mutagenesis, 99.8% reduced activity compared to the wild-type enzyme, 88% complementation of enzyme-deficient Escherichia coli strain SAS38X Homo sapiens

Molecular Weight [Da]

EC Number Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
1.3.3.4 51000
-
 2 * 51000, SDS-PAGE Homo sapiens

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
1.3.3.4 protoporphyrinogen-IX + 3 O2 Homo sapiens
-
 protoporphyrin-IX + 3 H2O2
-
 ?

Organism

EC Number Organism UniProt Comment Textmining
1.3.3.4 Homo sapiens
-
-
-

Reaction

EC Number Reaction Comment Organism Reaction ID
1.3.3.4 protoporphyrinogen IX + 3 O2 = protoporphyrin IX + 3 H2O2 residues G40, L85, G232, H281, V282, L295, V335, S350, L444, G453, L15, R38, L73, V84, D143, R152, L154, V158, R168, A172, V290, and G453 are essential for enzyme activity Homo sapiens
a

Specific Activity [micromol/min/mg]

EC Number Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
1.3.3.4 additional information
-
 activity of mutant enzymes, overview Homo sapiens
1.3.3.4 0.0023
-
 recombinant wild-type enzyme Homo sapiens

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
1.3.3.4 protoporphyrinogen-IX + 3 O2
-
 Homo sapiens protoporphyrin-IX + 3 H2O2
-
 ?

Subunits

EC Number Subunits Comment Organism
1.3.3.4 dimer 2 * 51000, SDS-PAGE Homo sapiens

Synonyms

EC Number Synonyms Comment Organism
1.3.3.4 PPOX
-
 Homo sapiens

Cofactor

EC Number Cofactor Comment Organism Structure
1.3.3.4 FAD 1 molecule per subunit Homo sapiens