ArticleMolecular genetics and metabolism2013
Glutathione imbalance in patients with X-linked adrenoleukodystrophy.
Article in Molecular genetics and metabolism, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 24 papers.
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Who cites it
24 citing papers in PubMed, 47 citations in OpenAlex.
- Mechanisms of Antioxidant Induction with High-Dose N-Acetylcysteine in Childhood Cerebral Adrenoleukodystrophy.CNS drugs · 2015Trial
- Inflammation and Immunomodulation in Cerebral X-linked Adrenoleukodystrophy: Review of Pathology and Interventions.Journal of child neurology · 2026Review
- Article
- Pathophysiology of X-Linked Adrenoleukodystrophy: Updates on Molecular Mechanisms.Journal of biotechnology and biomedicine · 2024Article
- A Phase 1 Study of Oral Vitamin DNeurology. Genetics · 2023Article
- Antioxidant Response in Human X-Linked Adrenoleukodystrophy Fibroblasts.Antioxidants (Basel, Switzerland) · 2022Article
- Isoprostanoid Plasma Levels Are Relevant to Cerebral Adrenoleukodystrophy Disease.Life (Basel, Switzerland) · 2022Article
- The Role of Oxidative Stress and Inflammation in X-Link Adrenoleukodystrophy.Frontiers in nutrition · 2022Review
- Review
- Article
- N-acetylcysteine Provides Cytoprotection in Murine Oligodendrocytes through Heme Oxygenase-1 Activity.Biomedicines · 2020Article
- Oxidative Stress Indexes for Diagnosis of Health or Disease in Humans.Oxidative medicine and cellular longevity · 2019Review
- Dendrimer-N-acetyl-L-cysteine modulates monophagocytic response in adrenoleukodystrophy.Annals of neurology · 2018Article
- Aberrant regulation of the GSK-3β/NRF2 axis unveils a novel therapy for adrenoleukodystrophy.EMBO molecular medicine · 2018Article
- Cysteamine revisited: repair of arginine to cysteine mutations.Journal of inherited metabolic disease · 2017Review
- Tauroursodeoxycholic bile acid arrests axonal degeneration by inhibiting the unfolded protein response in X-linked adrenoleukodystrophy.Acta neuropathologica · 2017Article
- Oxidative Stress in Patients with X-Linked Adrenoleukodystrophy.Cellular and molecular neurobiology · 2016Review
- Peroxisomes in brain development and function.Biochimica et biophysica acta · 2016Review
- Activation of sirtuin 1 as therapy for the peroxisomal disease adrenoleukodystrophy.Cell death and differentiation · 2015Article
- Detection and Quantification of Free Radicals in Peroxisomal Disorders: A Comparative Study with Oxidative Stress Parameters.Journal of clinical and diagnostic research : JCDR · 2015Article
Corrections and comments
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Authors and funding
8 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundX-linked adrenoleukodystrophy (X-ALD) is a genetic disorder of X-linked inheritance caused by a mutation in the ABCD1 gene which determines an accumulation of long-chain fatty acids in plasma and tissues. Recent evidence shows that oxidative stress may be a hallmark in the pathogenesis of X-ALD and glutathione plays an important role in the defense against free radicals. In this study we have analyzed glutathione homeostasis in lymphocytes of 14 patients with X-ALD and evaluated the balance between oxidized and reduced forms of glutathione, in order to define the role of this crucial redox marker in this condition.
methodsLymphocytes, plasma and erythrocytes were obtained from the whole blood of 14 subjects with X-ALD and in 30 healthy subjects. Total, reduced and protein-bound glutathione levels were measured in lymphocytes by HPLC analysis. Erythrocyte free glutathione and antioxidant enzyme activities, plasma thiols and carbonyl content were determined by spectrophotometric assays.
resultsA significant decrease of total and reduced glutathione was found in lymphocytes of patients, associated to high levels of all oxidized glutathione forms. A decline of free glutathione was particularly significant in erythrocytes. The increased oxidative stress in X-ALD was additionally confirmed by the decrease of plasma thiols and the high level of carbonyls.
conclusionOur results strongly support a role for oxidative stress in the pathophysiology of X-ALD and strengthen the importance of the balance among glutathione forms as a hallmark and a potential biomarker of the disease.
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