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Evidence of oxidative stress in peroxisomal disorders
Singapore medical journal ; : 608-614, 2012.
Artigo em Inglês | 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>
Assuntos
Texto completo: DisponíveL Índice: WPRIM (Pacífico Ocidental) Assunto principal: Linhagem / Superóxido Dismutase / Sangue / Biomarcadores / Estudos de Casos e Controles / Análise por Pareamento / Estresse Oxidativo / Consanguinidade / Transtornos Peroxissômicos / Egito Tipo de estudo: Estudo observacional Limite: Adolescente / Criança / Criança, pré-escolar / Humanos / Lactente País/Região como assunto: África Idioma: Inglês Revista: Singapore medical journal Ano de publicação: 2012 Tipo de documento: Artigo

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Texto completo: DisponíveL Índice: WPRIM (Pacífico Ocidental) Assunto principal: Linhagem / Superóxido Dismutase / Sangue / Biomarcadores / Estudos de Casos e Controles / Análise por Pareamento / Estresse Oxidativo / Consanguinidade / Transtornos Peroxissômicos / Egito Tipo de estudo: Estudo observacional Limite: Adolescente / Criança / Criança, pré-escolar / Humanos / Lactente País/Região como assunto: África Idioma: Inglês Revista: Singapore medical journal Ano de publicação: 2012 Tipo de documento: Artigo