Original Paper

Neurochemical Research

, Volume 31, Issue 9, pp 1103-1109

First online:

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

  • Pilar del HoyoAffiliated withDepartamento de Bioquímica—Investigación, Hospital Universitario Doce de Octubre
  • , Alberto García-RedondoAffiliated withDepartamento de Bioquímica—Investigación, Hospital Universitario Doce de Octubre
  • , Fernando de BustosAffiliated withServicio de Bioquímica, Hospital Nuestra Señora del Prado, Talavera de la Reina
  • , José Antonio MolinaAffiliated withServicio de Neurología, Hospital Universitario Doce de Octubre
  • , Youssef SayedAffiliated withDepartamento de Medicina-Neurología, Hospital “Príncipe de Asturias”, Universidad de Alcalá, Alcalá de Henares
  • , Hortensia Alonso-NavarroAffiliated withDepartamento de Medicina-Neurología, Hospital “Príncipe de Asturias”, Universidad de Alcalá, Alcalá de Henares
  • , Luis CaballeroAffiliated withServicio de Bioquímica, Hospital Nuestra Señora del Prado, Talavera de la Reina
  • , Joaquín ArenasAffiliated withDepartamento de Bioquímica—Investigación, Hospital Universitario Doce de Octubre
  • , Félix Javier Jiménez-JiménezAffiliated withDepartamento de Medicina-Neurología, Hospital “Príncipe de Asturias”, Universidad de Alcalá, Alcalá de Henares Email author 

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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.

Keywords

Huntington’ disease Oxidative stress Mitochondrial respiratory chain Glutathione-peroxidase Superoxide-dismutase Coenzyme Q10 Catalase Etiopathogenesis