Antioxidant agents have a different expression pattern in muscle fibers of patients with mitochondrial diseases

Acta Neuropathologica
Massimiliano FilostoGiuliano Tomelleri

Abstract

Respiratory chain dysfunction leads to reactive oxygen species (ROS) generation with following oxidative stress and cellular damage. A histochemical and immunohistochemical study was performed on muscle biopsies from 17 patients with mitochondrial disease [chronic progressive external ophthalmoplegia (CPEO), mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS), myoclonic epilepsy with ragged red fibers (MERRF)] to evaluate the expression pattern and location of manganese superoxide dismutase (MnSOD), copper-zinc superoxide dismutase (CuZnSOD) and reduced glutathione (GSH) in skeletal muscle fibers. Our data showed that: (1) MnSOD, CuZnSOD and GSH are expressed in fibers with respiratory chain deficiency; (2) the antioxidant induction is correlated with the degree of mitochondrial proliferation, but not with clinical phenotype, patients' age, duration of disease, biochemical defects or mitochondrial DNA abnormalities. In addition, we suggest that expression of MnSOD and GSH may be considered an initial, indirect sign of respiratory chain dysfunction because it is observed in the early stages of the disease.

Citations

May 24, 2011·Journal of Neurology·Massimiliano FilostoAlessandro Padovani
Oct 12, 2012·Journal of Inherited Metabolic Disease·Lorena Gallego-VillarLourdes R Desviat
Dec 19, 2003·Molecular Genetics and Metabolism·Ching-You LuYau-Huei Wei
Feb 19, 2009·Proceedings of the National Academy of Sciences of the United States of America·Kondala R AtkuriGregory M Enns
Dec 16, 2010·Molecular & Cellular Proteomics : MCP·Gaetano VattemiGiuliano Tomelleri
Feb 6, 2003·The Journal of Clinical Investigation·Eric A Schon, Giovanni Manfredi
Jun 16, 2015·Free Radical Biology & Medicine·Genki Hayashi, Gino Cortopassi
Apr 29, 2009·Biochimica Et Biophysica Acta·Yi-Shing MaYau-Huei Wei
Feb 6, 2007·Mitochondrion·Yasutoshi KogaToyojiro Matsuishi
Aug 14, 2012·Journal of Cellular Biochemistry·Eva RichardBelén Pérez
Aug 7, 2013·The International Journal of Biochemistry & Cell Biology·Lorenzo PolimenoAntonio Francavilla
Apr 16, 2013·Molecular Genetics and Metabolism·Anna PastoreFiorella Piemonte
May 4, 2017·Journal of Clinical Medicine·Gregory M Enns, Tina M Cowan
Jul 29, 2004·Clinical Science·Mikhail F AlexeyevGlenn L Wilson
Sep 4, 2004·International Journal of Immunopathology and Pharmacology·Q NiuP Boscolo

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