Journal of Neurology

, Volume 254, Issue 12, pp 1676–1683

Oxidative stress parameters in plasma of Huntington's disease patients, asymptomatic Huntington’s disease gene carriers and healthy subjects

A cross-sectional study
  • N. Klepac
  • M. Relja
  • R. Klepac
  • S. Hećimović
  • T. Babić
  • V. Trkulja
ORIGINAL COMMUNICATION

DOI: 10.1007/s00415-007-0611-y

Cite this article as:
Klepac, N., Relja, M., Klepac, R. et al. J Neurol (2007) 254: 1676. doi:10.1007/s00415-007-0611-y
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Abstract

Background

Animal data and postmortem studies suggest a role of oxidative stress in the Huntington's disease (HD), but in vivo human studies have been scarce.

Aim

To assess the presence of oxidative stress in HD patients and its occurrence relative to clinical symptoms.

Methods

Oxidative stress markers were determined in plasma of HD patients (n = 19), asymptomatic HD gene carriers (with > 38 CAG repeats) (n = 11) and their respective sex and agematched healthy controls (n = 47 and n = 22) in a cross-sectional study.

Results

With adjustment for age and sex, HD patients had higher plasma lipid peroxidation (LP) levels (ratio 1.20, 95% CI 1.09 to 1.32, p < 0.001) and lower reduced glutathione (GSH) levels (ratio 0.72, CI 0.55 to 0.94, p = 0.011) than their age and sex-matched controls. Although considerably younger, HD gene carriers did not differ from HD patients regarding LP and GSH levels, and had higher plasma LP (ratio 1.16, CI 1.02 to 1.32, p = 0.016) and lower GSH than their matched controls (ratio 0.73, CI 0.5 to 1.05). They had higher LP (ratio 1.18, CI 1.02 to 1.34, p = 0.019) and lower GSH (ratio 0.75, CI 0.51 to 1.11) than the healthy subjects matched to HD patients.

Conclusions

Oxidative stress is more pronounced in HD patients and asymptomatic HD gene carriers than in healthy subjects. Differences in plasma LP and GSH are in line with the brain findings in animal models of HD. Data suggest that oxidative stress occurs before the onset of the HD symptoms.

Key words

Huntington's diseaseoxidative stressUnified Huntington's Disease Rating Scale (UHDRS)lipid peroxidationreduced glutathione

Copyright information

© Steinkopff Verlag 2007

Authors and Affiliations

  • N. Klepac
    • 1
  • M. Relja
    • 1
  • R. Klepac
    • 2
  • S. Hećimović
    • 3
  • T. Babić
    • 1
  • V. Trkulja
    • 4
  1. 1.Dept. of NeurologyUniversity Clinical Hospital Center Zagreb, Zagreb University School of MedicineZagrebCroatia
  2. 2.Dept. of Biology ZagrebUniversity School of MedicineZagrebCroatia
  3. 3.Division of Molecular MedicineRuđer Bošković InstituteZagrebCroatia
  4. 4.Dept. of PharmacologyZagreb University School of MedicineZagrebCroatia