MDs (mitochondrial diseases) are a clinically heterogeneous group of disorders characterized by impairment of the respiratory chain function with altered oxidative phosphorylation. We tested the hypothesis that the function of vascular endothelium is affected by increased oxidative stress in MDs. A total of 12 patients with MDs and pair-matched controls were studied. Endothelial function was assessed by measuring FMD (flow-mediated vasodilation) of brachial and common femoral arteries. The test was repeated after vitamin C (500 mg, twice a day) and E (400 mg, once a day) supplementation for 30 days and 90 days after vitamin withdrawal. FMD was reduced in patients compared with controls [AUC/τ (time-averaged area under the curve) for the brachial artery, 1.05±0.24 compared with 4.19±0.59% respectively, P<0.001; AUC/τ for the femoral artery, 0.98±0.19 compared with 2.36±0.29% respectively, P=0.001; values are means±S.E.M.] and correlated (brachial artery) with plasma lactate (r=−0.63, P<0.01). Urinary 8-iso-PGF2α (8-iso-prostaglandin F2α) was higher in patients than controls (505.6±85.9 compared with 302.5±38.7 pg/mg of creatinine; P<0.05) and correlated with plasma lactate (r=0.70, P<0.05). Immunohistochemical analysis showed 8-iso-PGF2α staining in MD-affected striated muscle cells and in blood vessels in muscle biopsies of patients. Antioxidant vitamins transiently restored FMD in patients [ΔAUC/τ (change in AUC/τ) for the brachial artery, +1.38±0.49%, P<0.05; ΔAUC/τ for the femoral artery, +0.98±0.24%, P<0.01] but had no effect on FMD in controls (brachial artery, −1.3±0.63%; and common femoral artery, −0.58±0.30%), thus abolishing the differences between patients and controls. The results of the present study indicate that oxidative stress is increased and is, at least partly, responsible for endothelial dysfunction in MDs.
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November 22 2011
Endothelial dysfunction and increased oxidative stress in mitochondrial diseases
Pietro Minuz;
*Department of Medicine, Section of Internal Medicine, University of Verona, Verona, Italy
Correspondence: Professor Pietro Minuz (email [email protected]).
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Cristiano Fava;
Cristiano Fava
1
*Department of Medicine, Section of Internal Medicine, University of Verona, Verona, Italy
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Gaetano Vattemi;
Gaetano Vattemi
1
†Department of Neurological, Neuropsychological, Morphological and Movement Sciences, University of Verona, Verona, Italy
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Guido Arcaro;
Guido Arcaro
*Department of Medicine, Section of Internal Medicine, University of Verona, Verona, Italy
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Matteo Riccadonna;
Matteo Riccadonna
*Department of Medicine, Section of Internal Medicine, University of Verona, Verona, Italy
†Department of Neurological, Neuropsychological, Morphological and Movement Sciences, University of Verona, Verona, Italy
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Paola Tonin;
Paola Tonin
†Department of Neurological, Neuropsychological, Morphological and Movement Sciences, University of Verona, Verona, Italy
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Alessandra Meneguzzi;
Alessandra Meneguzzi
*Department of Medicine, Section of Internal Medicine, University of Verona, Verona, Italy
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Maurizio Degan;
Maurizio Degan
*Department of Medicine, Section of Internal Medicine, University of Verona, Verona, Italy
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Valeria Guglielmi;
Valeria Guglielmi
†Department of Neurological, Neuropsychological, Morphological and Movement Sciences, University of Verona, Verona, Italy
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Alessandro Lechi;
Alessandro Lechi
*Department of Medicine, Section of Internal Medicine, University of Verona, Verona, Italy
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Giuliano Tomelleri
Giuliano Tomelleri
†Department of Neurological, Neuropsychological, Morphological and Movement Sciences, University of Verona, Verona, Italy
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Publisher: Portland Press Ltd
Received:
April 20 2011
Revision Received:
September 26 2011
Accepted:
October 05 2011
Accepted Manuscript online:
October 05 2011
Online ISSN: 1470-8736
Print ISSN: 0143-5221
© The Authors Journal compilation © 2012 Biochemical Society
2012
Clin Sci (Lond) (2012) 122 (6): 289–299.
Article history
Received:
April 20 2011
Revision Received:
September 26 2011
Accepted:
October 05 2011
Accepted Manuscript online:
October 05 2011
Citation
Pietro Minuz, Cristiano Fava, Gaetano Vattemi, Guido Arcaro, Matteo Riccadonna, Paola Tonin, Alessandra Meneguzzi, Maurizio Degan, Valeria Guglielmi, Alessandro Lechi, Giuliano Tomelleri; Endothelial dysfunction and increased oxidative stress in mitochondrial diseases. Clin Sci (Lond) 1 March 2012; 122 (6): 289–299. doi: https://doi.org/10.1042/CS20110199
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