Oxidant–antioxidant imbalance in the erythrocytes of sporadic amyotrophic lateral sclerosis patients correlates with the progression of disease

Babu, G. Nagesh; Kumar, Alok; Chandra, Ramesh; Puri, Sunil K.; Singh, Ram Lakhan; Kalita, Jayantee; Misra, Usha Kant

doi:10.1016/j.neuint.2008.01.009

Cited by 91 publications

(63 citation statements)

References 35 publications

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“…Forced reduction of GSH levels in the G93A mutant hSOD1 mouse model, through the knockdown of ␥-glutamyl cysteine ligase modifier subunit (the rate-limiting enzyme in GSH synthesis) led to a decrease in life span, increased oxidative stress, and accelerated motor dysfunction (34). Furthermore, in sporadic ALS patients, erythrocyte GSH levels were found to be diminished, whereas lipid peroxidation levels were significantly increased compared with healthy age-matched controls (35). Despite this abundant and compelling evidence for a key role of GSH depletion within neurodegenerative diseases, neither mitochondrial GSH nor mitochondrial GSH transport mechanisms explicitly have been studied within models of these diseases.…”

Section: Discussionmentioning

confidence: 99%

Mitochondrial Glutathione Transport Is a Key Determinant of Neuronal Susceptibility to Oxidative and Nitrosative Stress

Wilkins

Kirchhof

Manning

et al. 2013

Journal of Biological Chemistry

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Background: Mitochondrial glutathione transport has not been extensively studied within the CNS. Results: Cerebellar neurons and astrocytes use distinct mechanisms of mitochondrial glutathione transport. Conclusion: Inhibition of a single mitochondrial glutathione transporter renders neurons more susceptible to oxidative and nitrosative stress. Significance: Mitochondrial glutathione transport is essential to protect neurons from oxidative and nitrosative stress conditions common to neurodegenerative diseases.

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Section: Discussionmentioning

confidence: 99%

Mitochondrial Glutathione Transport Is a Key Determinant of Neuronal Susceptibility to Oxidative and Nitrosative Stress

Wilkins

Kirchhof

Manning

et al. 2013

Journal of Biological Chemistry

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“…Indeed, it has been shown that the expression profile of antioxidant enzymes is altered in the affected tissues or cells of patients with mitochondrial diseases [35][36][37][38]. The protein expression and activity levels of antioxidant enzymes such as Mn-SOD were found to increase in affected tissues of patients with a specific mitochondrial disease, which was a response of cells to avoid oxidative damage of ROS released by defective mitochondria [35].…”

Section: Imbalanced Expression Of Antioxidant Enzymes In the Merrf Skmentioning

confidence: 99%

Mitochondrial DNA Mutation-Elicited Oxidative Stress, Oxidative Damage, and Altered Gene Expression in Cultured Cells of Patients with MERRF Syndrome

Wu¹,

Ma²,

Wu³

et al. 2010

Mol Neurobiol

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Myoclonic epilepsy and ragged-red fibers (MERRF) syndrome is a rare disorder characterized by myoclonus, muscle weakness, cerebellar ataxia, heart conduction block, and dementia. It has been documented that 80-90% of the patients with MERRF syndrome are caused by the A8344G mutation in the tRNA(Lys) gene of mitochondrial DNA (mtDNA). We and other investigators have reported that the mtDNA mutation results in not only inefficient generation of adenosine triphosphate but also increased production of reactive oxygen species (ROS) in cultured cells harboring A8344G mutation of mtDNA. In addition, we found an imbalance in the gene expression of antioxidant enzymes in the skin fibroblasts of MERRF patients. The mRNA, protein, and enzyme activity levels of manganese-superoxide dismutase were increased, but those of Cu,Zn-SOD, catalase, and glutathione peroxidase did not show significant changes. Recently, we showed that the excess ROS could damage voltage-dependent anion channel, prohibitin, Lon protease, and aconitase in the MERRF cells. Moreover, there was a dramatic increase in the gene expression and activity of matrix metalloproteinase 1, which may contribute to the cytoskeleton remodeling involved in the weakness and atrophy of muscle commonly seen in MERRF patients. Taken together, we suggest that mtDNA mutation-elicited oxidative stress, oxidative damage, and altered gene expression are involved in the pathogenesis and progression of MERRF syndrome.

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“…Oxidative stress markers have been found in nervous and peripheral tissues of familial and sporadic ALS patients . Lipid peroxidation in the erythrocytes of ALS patients was significantly increased with respect to controls (Babu et al, 2008). Recently described chronic, but moderate regenerative, haemolytic anaemia of aged SOD1-knockout mice is associated with erythrocytes modifications and sensitivity to both intra-and extra-vascular haemolysis (Starzyński et al, 2009).…”

Section: Point Mutation In Superoxide Dismutasementioning

confidence: 91%

Point Mutations That Reduce Erythrocyte Resistance to Oxidative Stress

Volosnikov¹,

Serebryakova²

2012

Point Mutation

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Oxidant–antioxidant imbalance in the erythrocytes of sporadic amyotrophic lateral sclerosis patients correlates with the progression of disease

Cited by 91 publications

References 35 publications

Mitochondrial Glutathione Transport Is a Key Determinant of Neuronal Susceptibility to Oxidative and Nitrosative Stress

Mitochondrial Glutathione Transport Is a Key Determinant of Neuronal Susceptibility to Oxidative and Nitrosative Stress

Mitochondrial DNA Mutation-Elicited Oxidative Stress, Oxidative Damage, and Altered Gene Expression in Cultured Cells of Patients with MERRF Syndrome

Point Mutations That Reduce Erythrocyte Resistance to Oxidative Stress

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