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Oxidative Medicine and Cellular Longevity
Volume 2016, Article ID 3187560, 9 pages
Research Article

Evidence for Detrimental Cross Interactions between Reactive Oxygen and Nitrogen Species in Leber’s Hereditary Optic Neuropathy Cells

1Department of Biochemical Sciences and Istituto Pasteur-Fondazione Cenci Bolognetti, Sapienza University of Rome, Italy
2CNR Institute of Molecular Biology and Pathology, 00185 Rome, Italy
3Faculty of Pharmacy and Biochemistry, University of Zagreb, 10000 Zagreb, Croatia
4Department of Clinical and Molecular Medicine, Sapienza University of Rome, 00185 Rome, Italy
5Centre for Experimental Neurological Therapies, S. Andrea Hospital-Site, Department of Neuroscience, Mental Health and Sensory Organs (NESMOS), Sapienza University of Rome, 00185 Rome, Italy

Received 10 August 2015; Revised 19 October 2015; Accepted 25 October 2015

Academic Editor: Liudmila Korkina

Copyright © 2016 Micol Falabella et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Here we have collected evidence suggesting that chronic changes in the NO homeostasis and the rise of reactive oxygen species bioavailability can contribute to cell dysfunction in Leber’s hereditary optic neuropathy (LHON) patients. We report that peripheral blood mononuclear cells (PBMCs), derived from a female LHON patient with bilateral reduced vision and carrying the pathogenic mutation 11778/ND4, display increased levels of reactive oxygen species (ROS) and reactive nitrogen species (RNS), as revealed by flow cytometry, fluorometric measurements of nitrite/nitrate, and 3-nitrotyrosine immunodetection. Moreover, viability assays with the tetrazolium dye MTT showed that lymphoblasts from the same patient are more sensitive to prolonged NO exposure, leading to cell death. Taken together these findings suggest that oxidative and nitrosative stress cooperatively play an important role in driving LHON pathology when excess NO remains available over time in the cell environment.