Neurochemical Research

, Volume 31, Issue 9, pp 1103–1109

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

  • Pilar del Hoyo
  • Alberto García-Redondo
  • Fernando de Bustos
  • José Antonio Molina
  • Youssef Sayed
  • Hortensia Alonso-Navarro
  • Luis Caballero
  • Joaquín Arenas
  • Félix Javier Jiménez-Jiménez
Original Paper

DOI: 10.1007/s11064-006-9110-2

Cite this article as:
del Hoyo, P., García-Redondo, A., de Bustos, F. et al. Neurochem Res (2006) 31: 1103. doi:10.1007/s11064-006-9110-2

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 

Copyright information

© Springer Science+Business Media, Inc. 2006

Authors and Affiliations

  • Pilar del Hoyo
    • 1
  • Alberto García-Redondo
    • 1
  • Fernando de Bustos
    • 2
  • José Antonio Molina
    • 3
  • Youssef Sayed
    • 4
  • Hortensia Alonso-Navarro
    • 4
  • Luis Caballero
    • 2
  • Joaquín Arenas
    • 1
  • Félix Javier Jiménez-Jiménez
    • 4
    • 5
  1. 1.Departamento de Bioquímica—InvestigaciónHospital Universitario Doce de OctubreMadridSpain
  2. 2.Servicio de Bioquímica, Hospital Nuestra Señora del PradoTalavera de la ReinaToledoSpain
  3. 3.Servicio de NeurologíaHospital Universitario Doce de OctubreMadridSpain
  4. 4.Departamento de Medicina-Neurología, Hospital “Príncipe de Asturias”Universidad de Alcalá, Alcalá de HenaresMadridSpain
  5. 5.MadridSpain

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