Antioxidants restore protoporphyrinogen oxidase in variegate porphyria patients. (20th April 2013)

• Record Type: Journal Article
• Authors: Ferrer, Miguel D.; Tauler, Pedro; Sureda, Antoni; Palacín, Clara; Tur, Josep A.; Pons, Antoni
• Is Part Of: European journal of clinical investigation. Volume 43:Number 7(2013:Jul.)
• Page Start: 668
• Page End: 678
• Publication Date: 2013-04-20
• Subjects: Pathology -- Periodicals; Medical research -- Periodicals; 616.075
• DOI: 10.1111/eci.12091
• Languages: English
• ISSNs: 0014-2972

Abstract

<abstract abstract-type="main" id="eci12091-abs-0001"> <title>Abstract</title> <sec id="eci12091-sec-0001" sec-type="section"> <title>Background</title> <p>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.</p> </sec> <sec id="eci12091-sec-0002" sec-type="section"> <title>Materials and methods</title> <p>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.</p> </sec> <sec id="eci12091-sec-0003" sec-type="section"> <title>Results</title> <p>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 H<sub>2</sub>O<sub>2</sub> 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.</p> </sec> <sec id="eci12091-sec-0004" sec-type="section"> <title>Conclusions</title> <p>Lymphocytes from VP patients show reduced PPOX expression and present a greater susceptibility to producing H<sub>2</sub>O<sub>2</sub> and impaired H<sub>2</sub>O<sub>2</sub> 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.</p> </sec> </abstract>