ArticleArchives of disease in childhood2002
Antioxidant enzymes in blood of patients with Friedreich's ataxia.
Article in Archives of disease in childhood, 2002. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.
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Who cites it
20 citing papers in PubMed, 63 citations in OpenAlex.
- Review
- Iron homeostasis and ferroptosis in human diseases: mechanisms and therapeutic prospects.Signal transduction and targeted therapy · 2024Review
- Mitochondrial and metabolic dysfunction in Friedreich ataxia: update on pathophysiological relevance and clinical interventions.Neuronal signaling · 2021Review
- An Overview of the Ferroptosis Hallmarks in Friedreich's Ataxia.Biomolecules · 2020Review
- Antioxidant Therapies and Oxidative Stress in Friedreich´s Ataxia: The Right Path or Just a Diversion?Antioxidants (Basel, Switzerland) · 2020Review
- Oxidative Stress in DNA Repeat Expansion Disorders: A Focus on NRF2 Signaling Involvement.Biomolecules · 2020Review
- Article
- Oxidative stress in inherited mitochondrial diseases.Free radical biology & medicine · 2015Review
- TORC1 Inhibition by Rapamycin Promotes Antioxidant Defences in a Drosophila Model of Friedreich's Ataxia.PloS one · 2015Article
- Friedreich's Ataxia: A Neuronal Point of View on the Oxidative Stress Hypothesis.Antioxidants (Basel, Switzerland) · 2014Review
- Frataxin deficiency leads to reduced expression and impaired translocation of NF-E2-related factor (Nrf2) in cultured motor neurons.International journal of molecular sciences · 2013Article
- Neurodegeneration in Friedreich's ataxia: from defective frataxin to oxidative stress.Oxidative medicine and cellular longevity · 2013Review
- Redox processes in neurodegenerative disease involving reactive oxygen species.Current neuropharmacology · 2012Article
- Friedreich's ataxia: the vicious circle hypothesis revisited.BMC medicine · 2011Article
- Iron-dependent functions of mitochondria--relation to neurodegeneration.Journal of neural transmission (Vienna, Austria : 1996) · 2011Review
- Mesenchymal stem cells restore frataxin expression and increase hydrogen peroxide scavenging enzymes in Friedreich ataxia fibroblasts.PloS one · 2011Article
- Friedreich ataxia: molecular mechanisms, redox considerations, and therapeutic opportunities.Antioxidants & redox signaling · 2010Review
- Altered lipid metabolism in a Drosophila model of Friedreich's ataxia.Human molecular genetics · 2010Article
- The pathogenesis of Friedreich ataxia and the structure and function of frataxin.Journal of neurology · 2009Review
- Friedreich ataxia-update on pathogenesis and possible therapies.Neurogenetics · 2004Review
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Authors and funding
12 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
BACKGROUND AND
aimsIncreased generation of reactive oxygen species and mitochondrial dysfunction may underlie the pathophysiology of Friedreich's ataxia, the most common inherited ataxia, due to GAA expansion in a gene coding for a mitochondrial protein (frataxin), implicated in the regulation of iron metabolism. Because iron overload would cause oxidative stress in Friedreich's ataxia, we investigated the enzyme antioxidant system in the blood of 14 patients by determining superoxide dismutase, glutathione peroxidase, and glutathione transferase catalytic activities. We also studied the glutathione S-transferase genotype polymorphism in order to evaluate its possible influence on enzyme activity.
methodsBlood samples were obtained from 14 unrelated patients with Friedreich's ataxia and 21 age matched healthy subjects. Antioxidant enzyme determinations were spectrophotometrically assayed using specific substrates; the glutathione S-transferase genotype polymorphism was analysed by endonuclease restriction mapping of exon 5 and 6 amplification products.
resultsThere was a significant elevation of the superoxide dismutase/glutathione peroxidase activity ratio (0.037 (0.01) v 0.025 (0.008) of controls) and an 83% rise of glutathione transferase specific activity (0.22 (0.1) v 0.12 (0.03) nmol/min/mg protein) in blood of patients with Friedreich's ataxia than in the controls. The genotype polymorphism of glutathione S-transferase enzyme did not show any relevant differences when compared to that of healthy subjects.
conclusionsData show an impairment in vivo of antioxidant enzymes in patients with Friedreich's ataxia and provide evidence of an increased sensitivity to oxidative stress, supporting a consistent role of free radical cytotoxicity in the pathophysiology of the disease.
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