Altered redox status in Cuban patients with spinocerebellar ataxia type 2

Guevara-García, Mariela; Valle, Lizette Gil del; Martı́nez-Sánchez, Gregorio; Velásquez-Pérez, Luis

doi:10.1016/j.biomag.2012.01.003

Cited by 3 publications

(10 citation statements)

References 37 publications

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“…This result partially agrees with a previous study conducted in SCA2 patients (4), and similar results have been obtained in cellular and mice models for Huntington’s disease (8, 25). …”

Section: Discussionsupporting

confidence: 93%

“…Additionally, in patients and cell models for spinocerebellar ataxia type 3, it has been shown that oxidative stress could be associated with a significant alteration of SOD and CAT enzymatic activities ( 22 , 23 ). Preliminary results also pointed to a role for oxidative stress as part of the SCA2 physiopathological processes, although contradictory results have been obtained regarding SOD3 and CAT enzymatic activities, probably due to limited sample sizes ( 4 , 13 ). More recently, an increase in SOD2 and CAT mRNA and protein levels in fibroblasts of SCA2 patients relative to controls was found, highlighting the significance of these enzymes in counteracting SCA2 neurodegenerative processes ( 24 ).…”

Section: Discussionmentioning

confidence: 99%

“…Preliminary evidences have been obtained supporting a role for these enzymes in SCA2 physiopathology ( 4 , 13 ). However, a deeper characterization of the influence of SOD and CAT enzymatic activities on the SCA2 clinical phenotype is needed to assess the potential value of these enzymes as disease biomarkers.…”

Section: Introductionmentioning

confidence: 96%

“…Little is known about the physiopathological mechanisms triggering SCA2 neurodegeneration. However, it has been suggested that oxidative stress might have an important role on the pathological processes ( 4 , 5 ). Oxidative stress has been pointed out as a pathological mechanism for additional polyglutamine disorders.…”

Section: Introductionmentioning

confidence: 99%

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Spinocerebellar Ataxia Type 2 Is Associated with the Extracellular Loss of Superoxide Dismutase but Not Catalase Activity

Almaguer-Gotay¹,

Almaguer-Mederos²,

Aguilera-Rodríguez³

et al. 2017

Front. Neurol.

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BackgroundSpinocerebellar ataxia type 2 (SCA2) is an inherited and still incurable neurodegenerative disorder. Evidence suggests that pro-oxidant agents as well as factors involved in antioxidant cellular defenses are part of SCA2 physiopathology.AimTo assess the influence of superoxide dismutase (SOD3) and catalase (CAT) enzymatic activities on the SCA2 syndrome.MethodClinical, molecular, and electrophysiological variables, as well as SOD3 and CAT enzymatic activities were evaluated in 97 SCA2 patients and in 64 age- and sex-matched control individuals.ResultsSpinocerebellar ataxia type 2 patients had significantly lower SOD3 enzymatic activity than the control group. However, there were no differences between patients and controls for CAT enzymatic activity. The effect size for the loss of patients’ SOD3 enzymatic activity was 0.342, corresponding to a moderate effect. SOD3 and CAT enzymatic activities were not associated with the CAG repeat number at the ATXN2 gene. SOD3 and CAT enzymatic activities did not show significant associations with the age at onset, severity score, or the studied electrophysiological markers.ConclusionThere is a reduced SOD3 enzymatic activity in SCA2 patients with no repercussion on the clinical phenotype.

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“…This result partially agrees with a previous study conducted in SCA2 patients (4), and similar results have been obtained in cellular and mice models for Huntington’s disease (8, 25). …”

Section: Discussionsupporting

confidence: 93%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 96%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Spinocerebellar Ataxia Type 2 Is Associated with the Extracellular Loss of Superoxide Dismutase but Not Catalase Activity

Almaguer-Gotay¹,

Almaguer-Mederos²,

Aguilera-Rodríguez³

et al. 2017

Front. Neurol.

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“…Measures of antioxidant-prooxidant balance in SCA2 patients suggest a significant increase in advanced oxidation protein products and peroxidation potential, as well as decrease in ferric reducing ability in plasma, GSH, and total hydroperoxides ( 113 ). Also, increased enzymatic activity was recently observed for the glutation S -transferase (GST) in a distinct cohort of Cuban patients ( 114 ).…”

Section: Biochemical Findingsmentioning

confidence: 99%

Spinocerebellar Ataxia Type 2: Clinicogenetic Aspects, Mechanistic Insights, and Management Approaches

2017

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Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant cerebellar ataxia that occurs as a consequence of abnormal CAG expansions in the ATXN2 gene. Progressive clinical features result from the neurodegeneration of cerebellum and extra-cerebellar structures including the pons, the basal ganglia, and the cerebral cortex. Clinical, electrophysiological, and imaging approaches have been used to characterize the natural history of the disease, allowing its classification into four distinct stages, with special emphasis on the prodromal stage, which is characterized by a plethora of motor and non-motor features. Neuropathological investigations of brain tissue from SCA2 patients reveal a widespread involvement of multiple brain systems, mainly cerebellar and brainstem systems. Recent findings linking ataxin-2 intermediate expansions to other neurodegenerative diseases such as amyotrophic lateral sclerosis have provided insights into the ataxin-2-related toxicity mechanism in neurodegenerative diseases and have raised new ethical challenges to molecular predictive diagnosis of SCA2. No effective neuroprotective therapies are currently available for SCA2 patients, but some therapeutic options such as neurorehabilitation and some emerging neuroprotective drugs have shown palliative benefits.

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