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

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.

journal_name

Acta Neuropathol

journal_title

Acta neuropathologica

authors

Filosto M,Tonin P,Vattemi G,Spagnolo M,Rizzuto N,Tomelleri G

doi

10.1007/s004010100455

subject

Has Abstract

pub_date

2002-03-01 00:00:00

pages

215-20

issue

3

eissn

0001-6322

issn

1432-0533

journal_volume

103

pub_type

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