Cloned (Comment) | Organism |
---|---|
DNA and amino acid sequence determination and analysis, expression of wild-type and mutant enzymes in Escherichia coli and as GFP-fusion proteins in COS-1 cells | Homo sapiens |
Protein Variants | Comment | Organism |
---|---|---|
I12T | naturally occurring mutation in a finnish variegate porphyria patient, genotype-phenotype analysis, highly reduced activity | Homo sapiens |
L401F | naturally occurring mutation in a finnish variegate porphyria patient, genotype-phenotype analysis, inactive mutant | Homo sapiens |
additional information | construction of frameshift, deletion and insertion mutants, construction of N-terminally truncated mutants, determination of subcellular localization in expressing COS-1 cells, genotype-phenotype analysis of naturally occurring mutations in a finnish variegate porphyria patients, overview | Homo sapiens |
R152C | naturally occurring mutation in a finnish variegate porphyria patient, genotype-phenotype analysis, highly reduced activity | Homo sapiens |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
mitochondrial inner membrane | analysis of mitochondrial targeting sequences of wild-type and mutant enzymes, overview | Homo sapiens | 5743 | - |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
protoporphyrinogen-IX + 3 O2 | Homo sapiens | the penultimate step in the heme biosynthesis, enzyme deficiency causes variegate porphyria | protoporphyrin-IX + 3 H2O2 | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | P50336 | healthy persons and finnish variegate porphyria patients | - |
Posttranslational Modification | Comment | Organism |
---|---|---|
additional information | analysis of mitochondrial targeting sequences of wild-type and mutant enzymes, overview | Homo sapiens |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
protoporphyrinogen-IX + 3 O2 | - |
Homo sapiens | protoporphyrin-IX + 3 H2O2 | - |
? | |
protoporphyrinogen-IX + 3 O2 | the penultimate step in the heme biosynthesis, enzyme deficiency causes variegate porphyria | Homo sapiens | protoporphyrin-IX + 3 H2O2 | - |
? |
Synonyms | Comment | Organism |
---|---|---|
PPOX | - |
Homo sapiens |
Cofactor | Comment | Organism | Structure |
---|---|---|---|
FAD | - |
Homo sapiens |