Oxidative stress biomarkers in mitochondrial myopathies, basally and after cysteine donor supplementation

Michelangelo Mancuso; Daniele Orsucci; Annalisa LoGerfo; Anna Rocchi; Lucia Petrozzi; Claudia Nesti; Fabio Galetta; Gino Santoro; Luigi Murri; Gabriele Siciliano

doi:10.1007/s00415-009-5409-7

Oxidative stress biomarkers in mitochondrial myopathies, basally and after cysteine donor supplementation

Michelangelo Mancuso, Daniele Orsucci, Annalisa LoGerfo, Anna Rocchi, Lucia Petrozzi, Claudia Nesti, Fabio Galetta, Gino Santoro, Luigi Murri, Gabriele Siciliano

Fondazione Stella Maris

Research output: Contribution to journal › Article › peer-review

Abstract

Mitochondrial diseases are due to impairment of the mitochondrial respiratory chain. A plausible pathogenic mechanism leading to cellular dysfunction and phenotypic expression is oxidative stress, but there are surprisingly few clinical studies on this subject. Glutathione (GSH) deficiency has been reported in mitochondrial diseases, and the biosynthesis of glutathione depends on cysteine availability. We have examined oxidative stress biomarkers [advanced oxidation protein products (AOPP) and ferric reducing antioxidant power (FRAP)] in blood samples from 27 patients and 42 controls. AOPP levels were greater in patients than in controls (P value

Original language	English
Pages (from-to)	774-781
Number of pages	8
Journal	Journal of Neurology
Volume	257
Issue number	5
DOIs	https://doi.org/10.1007/s00415-009-5409-7
Publication status	Published - May 2010

Keywords

Mitochondrial disorders
Mitochondrial myopathy
mtDNA
Reactive oxygen species
ROS

ASJC Scopus subject areas

Clinical Neurology
Neurology

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10.1007/s00415-009-5409-7

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abstract = "Mitochondrial diseases are due to impairment of the mitochondrial respiratory chain. A plausible pathogenic mechanism leading to cellular dysfunction and phenotypic expression is oxidative stress, but there are surprisingly few clinical studies on this subject. Glutathione (GSH) deficiency has been reported in mitochondrial diseases, and the biosynthesis of glutathione depends on cysteine availability. We have examined oxidative stress biomarkers [advanced oxidation protein products (AOPP) and ferric reducing antioxidant power (FRAP)] in blood samples from 27 patients and 42 controls. AOPP levels were greater in patients than in controls (P value ",

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AU - Mancuso, Michelangelo

AU - Orsucci, Daniele

AU - LoGerfo, Annalisa

AU - Rocchi, Anna

AU - Petrozzi, Lucia

AU - Nesti, Claudia

AU - Galetta, Fabio

AU - Santoro, Gino

AU - Murri, Luigi

AU - Siciliano, Gabriele

PY - 2010/5

Y1 - 2010/5

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AB - Mitochondrial diseases are due to impairment of the mitochondrial respiratory chain. A plausible pathogenic mechanism leading to cellular dysfunction and phenotypic expression is oxidative stress, but there are surprisingly few clinical studies on this subject. Glutathione (GSH) deficiency has been reported in mitochondrial diseases, and the biosynthesis of glutathione depends on cysteine availability. We have examined oxidative stress biomarkers [advanced oxidation protein products (AOPP) and ferric reducing antioxidant power (FRAP)] in blood samples from 27 patients and 42 controls. AOPP levels were greater in patients than in controls (P value

KW - Mitochondrial disorders

KW - Mitochondrial myopathy

KW - mtDNA

KW - Reactive oxygen species

KW - ROS

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Oxidative stress biomarkers in mitochondrial myopathies, basally and after cysteine donor supplementation

Abstract

Keywords

ASJC Scopus subject areas

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