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Literature summary for 1.3.3.4 extracted from

  • Ferrer, M.D.; Tauler, P.; Sureda, A.; Palacin, C.; Tur, J.A.; Pons, A.
    Antioxidants restore protoporphyrinogen oxidase in variegate porphyria patients (2013), Eur. J. Clin. Invest., 43, 668-678 .
    View publication on PubMed

Cloned(Commentary)

Cloned (Comment) Organism
gene PPOX, relative real-time RT-PCR enzyme expression analysis Homo sapiens

Organism

Organism UniProt Comment Textmining
Homo sapiens P50336
-
-

Source Tissue

Source Tissue Comment Organism Textmining
lymphocyte from healthy women and porphyria (VP) patient women, enzyme PPOX activity is reduced in the latter Homo sapiens
-

Synonyms

Synonyms Comment Organism
PPOX
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Homo sapiens

Temperature Optimum [°C]

Temperature Optimum [°C] Temperature Optimum Maximum [°C] Comment Organism
37
-
assay at Homo sapiens

pH Optimum

pH Optimum Minimum pH Optimum Maximum Comment Organism
7.4
-
assay at Homo sapiens

Expression

Organism Comment Expression
Homo sapiens antioxidant vitamins increase PPOX expression in variegate porphyria patients up

General Information

General Information Comment Organism
malfunction antioxidants restore protoporphyrinogen oxidase in variegate porphyria patients showing accumulation of heme precursors and a low rate of heme biosynthesis. Lymphocytes from variegate porphyria patients show reduced PPOX expression and present a greater susceptibility to producing H2O2 and impaired H2O2 detoxifying mechanisms. Supplementation with vitamins E and C restores PPOX expression in variegate porphyria patients and enhances glutathione reductase (GRd) and superoxide dismutase (SOD) activities. Phenotype, overview Homo sapiens