Evidence of oxidative stress in peroxisomal disorders
Singapore medical journal
;
: 608-614, 2012.
Article
Dans Anglais
| WPRIM
| ID: wpr-249660
ABSTRACT
<p><b>INTRODUCTION</b>Peroxisomal disorders are subdivided into peroxisome biogenesis disorders (PBDs) and single peroxisomal enzyme deficiency. Many peroxisomal diseases exhibit excessive oxidative stress, leading to neurological alterations and dysfunction. Peroxisomes use oxygen in oxidative reactions that generate hydrogen peroxide. This study aimed to investigate various oxidative stress parameters in patients suffering from peroxisomal disorders.</p><p><b>METHODS</b>A total of 20 patients with peroxisomal disorders, aged six months to 13 years (mean age 5.9 ± 3.2 years), were compared to 14 healthy controls. All individuals were subjected to full history-taking, including a three-generation pedigree analysis concerning parental consanguinity and similarly affected members in the family, with meticulous clinical examination to detect any malformation or anomaly. Estimation of very-long-chain fatty acids and phytanic acid was done to verify the diagnosis. Brain magnetic resonance imaging, electroencephalogram, visual evoked potential, auditory potential and plain radiography were conducted to assess the pathological condition of the patients. Oxidative stress parameters, including nitric oxide (NO), malondialdehyde (MDA) and superoxide dismutase (SOD), were estimated in both the patients and controls.</p><p><b>RESULTS</b>Significant increases in both MDA and NO were found in patients with PBDs. It was also demonstrated that SOD was significantly lower in patients with PDB than the controls.</p><p><b>CONCLUSION</b>This study sheds more light on the link between oxidative stress and peroxisomal disorders, as oxidative stress may be a hallmark of peroxisomal disorders. Consequently, one of the useful neuronal rescue strategies could be treatment with antioxidant agents in addition to other lines of treatments.</p>
Texte intégral:
Disponible
Indice:
WPRIM (Pacifique occidental)
Sujet Principal:
Pedigree
/
Superoxide dismutase
/
Sang
/
Marqueurs biologiques
/
Études cas-témoins
/
Analyse appariée
/
Stress oxydatif
/
Consanguinité
/
Maladies péroxysomiales
/
Égypte
Type d'étude:
Étude observationnelle
Limites du sujet:
Adolescent
/
Enfant
/
Enfant d'âge préscolaire
/
Humains
/
Bébé
Pays comme sujet:
Afrique
langue:
Anglais
Texte intégral:
Singapore medical journal
Année:
2012
Type:
Article
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