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Literature summary for 1.3.3.4 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
Search for more literature for 1.3.3.4

Cloned(Commentary)

Cloned (Comment) Organism
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

Protein Variants Comment Organism
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
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
del281H site-directed mutagenesis, inactive mutant, no complementation of enzyme-deficient Escherichia coli strain SAS38X Homo sapiens
G232R site-directed mutagenesis, inactive mutant, no complementation of enzyme-deficient Escherichia coli strain SAS38X Homo sapiens
G40E site-directed mutagenesis, inactive mutant, no complementation of enzyme-deficient Escherichia coli strain SAS38X Homo sapiens
G453R site-directed mutagenesis, nearly inactive mutant, but 89% complementation of enzyme-deficient Escherichia coli strain SAS38X Homo sapiens
G453V site-directed mutagenesis, inactive mutant, no complementation of enzyme-deficient Escherichia coli strain SAS38X Homo sapiens
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
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
L295P site-directed mutagenesis, inactive mutant, no complementation of enzyme-deficient Escherichia coli strain SAS38X Homo sapiens
L444P site-directed mutagenesis, inactive mutant, no complementation of enzyme-deficient Escherichia coli strain SAS38X Homo sapiens
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
L85P site-directed mutagenesis, inactive mutant, no complementation of enzyme-deficient Escherichia coli strain SAS38X Homo sapiens
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
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
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
S350P site-directed mutagenesis, inactive mutant, no complementation of enzyme-deficient Escherichia coli strain SAS38X Homo sapiens
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
V282D site-directed mutagenesis, inactive mutant, no complementation of enzyme-deficient Escherichia coli strain SAS38X Homo sapiens
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
V335G site-directed mutagenesis, inactive mutant, no complementation of enzyme-deficient Escherichia coli strain SAS38X Homo sapiens
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]

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

Natural Substrates/ Products (Substrates)

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

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Reaction

Reaction Comment Organism Reaction ID
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]

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

Substrates and Products (Substrate)

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

Subunits

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

Synonyms

Synonyms Comment Organism
PPOX
-
 Homo sapiens

Cofactor

Cofactor Comment Organism Structure
FAD 1 molecule per subunit Homo sapiens