Redox Imbalance Associates with Clinical Worsening in Spinocerebellar Ataxia Type 2.

Autor: Dennis AG; Center for the Investigation and Rehabilitation of Hereditary Ataxias (CIRAH), Holguín, Cuba.; University of Medical Sciences of Holguín, Cuba., Almaguer-Mederos LE; Center for the Investigation and Rehabilitation of Hereditary Ataxias (CIRAH), Holguín, Cuba.; University of Medical Sciences of Holguín, Cuba., Raúl RA; Center for the Investigation and Rehabilitation of Hereditary Ataxias (CIRAH), Holguín, Cuba.; University of Medical Sciences of Holguín, Cuba., Roberto RL; Center for the Investigation and Rehabilitation of Hereditary Ataxias (CIRAH), Holguín, Cuba., Luis VP; Center for the Investigation and Rehabilitation of Hereditary Ataxias (CIRAH), Holguín, Cuba.; Cuban Academy of Sciences, Cuba., Dany CA; Center for the Investigation and Rehabilitation of Hereditary Ataxias (CIRAH), Holguín, Cuba.; University of Medical Sciences of Holguín, Cuba., Yanetza GZ; Center for the Investigation and Rehabilitation of Hereditary Ataxias (CIRAH), Holguín, Cuba.; University of Medical Sciences of Holguín, Cuba., Yaimeé VM; Center for the Investigation and Rehabilitation of Hereditary Ataxias (CIRAH), Holguín, Cuba., Annelié ED; Center for the Investigation and Rehabilitation of Hereditary Ataxias (CIRAH), Holguín, Cuba., Arnoy PA; Center for the Investigation and Rehabilitation of Hereditary Ataxias (CIRAH), Holguín, Cuba., Reydenis TV; Center for the Investigation and Rehabilitation of Hereditary Ataxias (CIRAH), Holguín, Cuba.
Jazyk: angličtina
Zdroj: Oxidative medicine and cellular longevity [Oxid Med Cell Longev] 2021 Feb 19; Vol. 2021, pp. 9875639. Date of Electronic Publication: 2021 Feb 19 (Print Publication: 2021).
DOI: 10.1155/2021/9875639
Abstrakt: Background: Spinocerebellar ataxia type 2 (SCA2) is a neurodegenerative disease presenting with redox imbalance. However, the nature and implications of redox imbalance in SCA2 physiopathology have not been fully understood.
Objective: The objective of this study is to assess the redox imbalance and its association with disease severity in SCA2 mutation carriers.
Methods: A case-control study was conducted involving molecularly confirmed SCA2 patients, presymptomatic individuals, and healthy controls. Several antioxidant parameters were assessed, including serum thiol concentration and the superoxide dismutase, catalase, and glutathione S-transferase enzymatic activities. Also, several prooxidant parameters were evaluated, including thiobarbituric acid-reactive species and protein carbonyl concentrations. Damage, protective, and OXY scores were computed. Clinical correlates were established.
Results: Significant differences were found between comparison groups for redox markers, including protein carbonyl concentration ( F = 3.30; p = 0.041), glutathione S-transferase activity ( F = 4.88; p = 0.009), and damage ( F = 3.20; p = 0.045), protection ( F = 12.75; p < 0.001), and OXY ( F = 7.29; p = 0.001) scores. Protein carbonyl concentration was positively correlated with CAG repeat length ( r = 0.27; p = 0.022), while both protein carbonyl concentration ( r = -0.27; p = 0.018) and OXY score ( r = -0.25; p = 0.013) were inversely correlated to the disease duration. Increasing levels of antioxidants and decreasing levels of prooxidant parameters were associated with clinical worsening.
Conclusions: There is a disruption of redox balance in SCA2 mutation carriers which depends on the disease stage. Besides, redox changes associate with markers of disease severity, suggesting a link between disruption of redox balance and SCA2 physiopathology.
Competing Interests: The authors declare that they have no conflicts of interest.
(Copyright © 2021 Almaguer-Gotay Dennis et al.)
Databáze: MEDLINE
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