title: Enzyme antioxidant defences and oxidative damage in red blood cells of variegate porphyria patients.
abstract: Variegate porphyria is the result of decreased protoporphyrinogen oxidase (PPOX) activity, the penultimate enzyme of haem biosynthesis. Haem precursors can produce free radicals and activate oxygen-inducing oxidative stress. Our aim was to analyse the effects of variegate porphyria on haemoglobin levels, antioxidant enzyme activities and oxidative damage in circulating erythrocytes. Twelve women affected by variegate porphyria and 12 control healthy women participated in the study. Women affected by variegate porphyria presented reduced PPOX content and delta-aminolevulinic acid dehydratase activity in erythrocytes. Haemoglobin content and mean corpuscular volume were higher in the porphyric group. Erythrocyte glutathione reductase and superoxide dismutase activities and catalase content were higher in porphyric women, although MDA levels were also higher in the erythrocytes of the porphyric group. In conclusion, the determination of PPOX could be a useful method to detect variegate porphyria. Despite having higher antioxidant defences, erythrocytes of porphyric women have greater oxidative damage and higher corpuscular volume, which are both indices of a situation of higher oxidative stress.
authors.affiliation: Laboratori de Ciències de l'Activitat Física, Departament de Biologia Fonamental i Ciències de la Salut, Grup de Nutrició Comunitaria i Estrés Oxidatiu, IUNICS, Universitat de les Illes Balears, Palma de Mallorca, Illes Balears, Spain.
authors.forename: Miguel D
authors.initials: MD
authors.lastname: Ferrer
authors.forename: Pedro
authors.initials: P
authors.lastname: Tauler
authors.forename: Antoni
authors.initials: A
authors.lastname: Sureda
authors.forename: Dora
authors.initials: D
authors.lastname: Romaguera
authors.forename: Isabel
authors.initials: I
authors.lastname: Llompart
authors.forename: Clara
authors.initials: C
authors.lastname: Palacin
authors.forename: Jaume
authors.initials: J
authors.lastname: Orfila
authors.forename: Josep A
authors.initials: JA
authors.lastname: Tur
authors.forename: Antoni
authors.initials: A
authors.lastname: Pons
mesh_terms: D000975:Antioxidants / Q000378:metabolism*; D002374:Catalase / Q000097:blood; D004912:Erythrocytes / Q000166:cytology / Q000201:enzymology; D005260:Female; D005980:Glutathione Reductase / Q000097:blood; D006454:Hemoglobins / Q000378:metabolism; D006801:Humans; D018384:Oxidative Stress; D000623:Porphobilinogen Synthase / Q000097:blood; D046350:Porphyria, Variegate / Q000378:metabolism* / Q000503:physiopathology; D050768:Protoporphyrinogen Oxidase / Q000097:blood*
chemical_list: D000975:Antioxidants; D006454:Hemoglobins; D002374:Catalase; D050768:Protoporphyrinogen Oxidase; D005980:Glutathione Reductase; D000623:Porphobilinogen Synthase