title: Antioxidants restore protoporphyrinogen oxidase in variegate porphyria patients.
abstract: BACKGROUND
  Variegate porphyria (VP) is the result of decreased protoporphyrinogen oxidase (PPOX) activity and results in the accumulation of porphyrins and porphyrin precursors. Our aims were to analyse the basal antioxidant defences and oxidative damage markers and the effects of a diet supplementation with vitamins E and C on the oxidant/antioxidant status and PPOX gene expression in lymphocytes of variegate porphyria (VP) patients.
  
  
  METHODS
  Twelve women affected by VP and 12 control women participated in a randomized and double-blind crossover study. Each participant took either 50 mg/day vitamin E and 150 mg/day vitamin C or a placebo for 6 months.
  
  
  RESULTS
  Lymphocyte PPOX gene expression, together with catalase and glutathione peroxidase activities, was reduced in VP women. No differences were observed in the levels of malondialdehyde and protein carbonyl derivatives. Stimulated lymphocyte H2 O2 production was higher in porphyric women. Supplementation with antioxidant vitamins increased PPOX expression in VP patients. Glutathione reductase (GRd) and superoxide dismutase (SOD) activities were higher in the treatment groups.
  
  
  CONCLUSIONS
  Lymphocytes from VP patients show reduced PPOX expression and present a greater susceptibility to producing H2 O2 and impaired H2 O2 detoxifying mechanisms. Supplementation with vitamins E and C restores PPOX expression in VP patients and enhances GRd and SOD activity, suggesting the potential benefits of a diet rich in vitamins E and C in these patients.
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, 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: Clara
authors.initials: C
authors.lastname: Palacín
authors.forename: Josep A
authors.initials: JA
authors.lastname: Tur
authors.forename: Antoni
authors.initials: A
authors.lastname: Pons
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000975:Antioxidants / Q000627:therapeutic use*; D001205:Ascorbic Acid / Q000627:therapeutic use*; D002374:Catalase / Q000097:blood; D018592:Cross-Over Studies; D019587:Dietary Supplements; D004311:Double-Blind Method; D005260:Female; D015870:Gene Expression; D005980:Glutathione Reductase / Q000097:blood; D006801:Humans; D006861:Hydrogen Peroxide / Q000097:blood; D008214:Lymphocytes / Q000201:enzymology; D008315:Malondialdehyde / Q000378:metabolism; D008875:Middle Aged; D046350:Porphyria, Variegate / Q000097:blood / Q000188:drug therapy*; D050768:Protoporphyrinogen Oxidase / Q000235:genetics / Q000378:metabolism*; D012333:RNA, Messenger; D060888:Real-Time Polymerase Chain Reaction; D013482:Superoxide Dismutase / Q000097:blood; D014810:Vitamin E / Q000627:therapeutic use*
chemical_list: D000975:Antioxidants; D012333:RNA, Messenger; D014810:Vitamin E; D008315:Malondialdehyde; D006861:Hydrogen Peroxide; D002374:Catalase; D013482:Superoxide Dismutase; D050768:Protoporphyrinogen Oxidase; D005980:Glutathione Reductase; D001205:Ascorbic Acid