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Oxidative Stress in Skin Fibroblasts Cultures of Patients with Huntington’s Disease

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Abstract

Oxidative stress and mitochondrial dysfunction should play a role in the neurodegeneration in Huntington’s disease (HD). The most consistent finding is decreased activity of the mitochondrial complexes II/III and IV of the respiratory chain in the striatum. We assessed enzymatic activities of respiratory chain enzymes and other enzymes involved in oxidative processes in skin fibroblasts cultures of patients with HD.

We studied respiratory chain enzyme activities, activities of total, Cu/Zn- and Mn-superoxide-dismutase, glutathione-peroxidase (GPx) and catalase, and coenzyme Q10 (CoQ10) levels in skin fibroblasts cultures from 13 HD patients and 13 age- and sex-matched healthy controls.

When compared with controls, HD patients showed significantly lower specific activities for catalase corrected by protein concentrations (P < 0.01). Oxidized, reduced and total CoQ10 levels (both corrected by citrate synthase (CS) and protein concentrations), and activities of total, Cu/Zn- and Mn-superoxide-dismutase, and gluthatione-peroxidase, did not differ significantly between HD-patients and control groups. Values for enzyme activities in the HD group did not correlate with age at onset and of the disease and with the CAG triplet repeats.

The primary finding of this study was the decreased activity of catalase in HD patients, suggesting a possible contribution of catalase, but not of other enzymes related with oxidative stress, to the pathogenesis of this disease.

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Acknowledgment

This work was supported in part by the grant of the Fondo de Investigaciones Sanitarias 99/0518.

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Authors and Affiliations

  1. Departamento de Bioquímica—Investigación, Hospital Universitario Doce de Octubre, Madrid, Spain

    Pilar del Hoyo, Alberto García-Redondo & Joaquín Arenas

  2. Servicio de Bioquímica, Hospital Nuestra Señora del Prado, Talavera de la Reina, Toledo, Spain

    Fernando de Bustos & Luis Caballero

  3. Servicio de Neurología, Hospital Universitario Doce de Octubre, Madrid, Spain

    José Antonio Molina

  4. Departamento de Medicina-Neurología, Hospital “Príncipe de Asturias”, Universidad de Alcalá, Alcalá de Henares, Madrid, Spain

    Youssef Sayed, Hortensia Alonso-Navarro & Félix Javier Jiménez-Jiménez

  5. C/Marroquina 14, 3° B, 28030, Madrid, Spain

    Félix Javier Jiménez-Jiménez

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  1. Pilar del Hoyo
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Correspondence to Félix Javier Jiménez-Jiménez.

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del Hoyo, P., García-Redondo, A., de Bustos, F. et al. Oxidative Stress in Skin Fibroblasts Cultures of Patients with Huntington’s Disease. Neurochem Res 31, 1103–1109 (2006). https://doi.org/10.1007/s11064-006-9110-2

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  • DOI: https://doi.org/10.1007/s11064-006-9110-2

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