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Titolo:
Apoptosis and ROS detoxification enzymes correlate with cytochrome c oxidase deficiency in mitochondrial encephalomyopathies
Autore:
Di Giovanni, S; Mirabella, M; Papacci, M; Odoardi, F; Silvestri, G; Servidei, S;
Indirizzi:
Catholic Univ Sacred Heart, Inst Neurol, I-00168 Rome, Italy Catholic UnivSacred Heart Rome Italy I-00168 eurol, I-00168 Rome, Italy
Titolo Testata:
MOLECULAR AND CELLULAR NEUROSCIENCE
fascicolo: 4, volume: 17, anno: 2001,
pagine: 696 - 705
SICI:
1044-7431(200104)17:4<696:AARDEC>2.0.ZU;2-9
Fonte:
ISI
Lingua:
ENG
Soggetto:
PROGRAMMED CELL-DEATH; REACTIVE OXYGEN; OXIDATIVE STRESS; SUPEROXIDE-DISMUTASE; INDUCE APOPTOSIS; MUSCLE; RADICALS; DISEASE;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
26
Recensione:
Indirizzi per estratti:
Indirizzo: Di Giovanni, S Catholic Univ Sacred Heart, Inst Neurol, Largo A Gemelli 8,I-00168 Rome, Italy Catholic Univ Sacred Heart Largo A Gemelli 8 Rome Italy I-00168
Citazione:
S. Di Giovanni et al., "Apoptosis and ROS detoxification enzymes correlate with cytochrome c oxidase deficiency in mitochondrial encephalomyopathies", MOL CELL NE, 17(4), 2001, pp. 696-705

Abstract

The aim of this work was to investigate in muscle the role of apoptosis and of oxidative stress in mitochondrial disorders with dysfunction of respiratory chain. In patients with cytochrome c oxidase deficiency (COX) we found a variable number of myofibers with apoptotic nuclei that matched with the level of enzymatic reduction and roughly correlated with muscle weakness. In parallel, a positive immunostaining for apoptosis-related proteins and Mn and Cu/Zn superoxide dismutase (SOD) were mostly localized in COX-negative fibers. Moreover, glutathione peroxidase activity was increased in muscles with high number of SOD-positive myofibers and prominent apoptotic features. No signs of apoptosis were observed in patients with deficiencies of complexes I and II and without muscle weakness. These data suggest that apoptosis along with increased ROS production, revealed by anti-oxidant enzymesoverexpression, may play an important role in the pathophysiology of mitochondrial diseases associated with COX deficiency.

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Documento generato il 07/04/20 alle ore 22:54:06