Falabella 2015 Oxid Med Cell Longev
|Falabella M, Forte E, Magnifico MC, Santini P, Arese M, Giuffrè A, Radić K, Chessa L, Coarelli G, Buscarinu MC, Mechelli R, Salvetti M, Sarti P (2015) Evidence for detrimental cross interactions between reactive oxygen and nitrogen species in Leber’s hereditary optic neuropathy cells. Oxid Med Cell Longev 3187560.|
Abstract: 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 (ROS) and nitrogen (RNS) species, 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.
• Keywords: Nitric oxide, LHON, Oxidative and nitrosative stress, PBMC
• O2k-Network Lab: IT Roma Sarti P
Labels: MiParea: Respiration, Patients Pathology: Other Stress:Oxidative stress;RONS Organism: Human Tissue;cell: Blood cells, Lymphocyte Preparation: Intact cells
Regulation: ATP, ATP production Coupling state: ROUTINE Pathway: ROX HRR: Oxygraph-2k