Oxidative stress in fibroblasts from patients with pseudoxanthoma elasticum: possible role in the pathogenesis of clinical manifestations (Articolo in rivista)

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Label
  • Oxidative stress in fibroblasts from patients with pseudoxanthoma elasticum: possible role in the pathogenesis of clinical manifestations (Articolo in rivista) (literal)
Anno
  • 2006-01-01T00:00:00+01:00 (literal)
Alternative label
  • Pasquali-Ronchetti, I; Garcia-Fernandez, MI; Boraldi, F; Quaglino, D; Gheduzzi, D; Paolinelli, CD; Tiozzo, R; Bergamini, S; Ceccarelli, D; Muscatello, U (2006)
    Oxidative stress in fibroblasts from patients with pseudoxanthoma elasticum: possible role in the pathogenesis of clinical manifestations
    in Journal of pathology (Print)
    (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#autori
  • Pasquali-Ronchetti, I; Garcia-Fernandez, MI; Boraldi, F; Quaglino, D; Gheduzzi, D; Paolinelli, CD; Tiozzo, R; Bergamini, S; Ceccarelli, D; Muscatello, U (literal)
Pagina inizio
  • 54 (literal)
Pagina fine
  • 61 (literal)
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  • 208 (literal)
Rivista
Note
  • ISI Web of Science (WOS) (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#affiliazioni
  • Univ Modena & Reggio Emilia, Dept Biomed Sci, I-41100 Modena, Italy; Univ Malaga, Sch Med, Dept Human Physiol, E-29080 Malaga, Spain; Univ Modena & Reggio Emilia, INFM, Natl Res Ctr Nanostruct & Biosyst Sufaces, I-41100 Modena, Italy (literal)
Titolo
  • Oxidative stress in fibroblasts from patients with pseudoxanthoma elasticum: possible role in the pathogenesis of clinical manifestations (literal)
Abstract
  • Pseudoxanthoma elasticum (PXE) is a genetic disease characterized by calcification and fragmentation of elastic fibres of the skin, cardiovascular system and eye, caused by mutations of the ABCC6 gene, which encodes the membrane transporter MRP6. The pathogenesis of the lesions is unknown. Based on studies of similar clinical and histopathological damage present in haemolytic disorders, our working hypothesis is that PXE lesions may result from chronic oxidative stress occurring in PXE cells as a consequence of MRP6 deficiency. Our results show that PXE fibroblasts suffer from mild chronic oxidative stress due to the imbalance between production and degradation of oxidant species. The findings also show that this imbalance results, at least in part, from the loss of mitochondrial membrane potential (Delta Psi(m)) with overproduction of H2O2. Whether mitochondrial dysfunction is the main factor responsible for the oxidative stress in PXE cells remains to be elucidated. However, mild chronic generalized oxidative stress could explain the great majority of structural and biochemical alterations already reported in PXE. Copyright (c) 2005 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd. (literal)
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