[The molecular bases of Huntington's disease: the role played by oxidative stress].
BACKGROUND
Huntington's disease is a neurodegenerative, autosomal dominant disease that mainly affects the basal ganglia. The disorder is caused by mutation in the gene encoding the huntingtin protein (Htt), producing intracellular aggregates. The adult form (chorea with dementia) is the most frequent. It is characterized by unpredictable, spasmodic, involuntary and constant movements, mostly associated with psychiatric and cognitive alterations.


METHODS
The main characteristics of Huntington's disease are: neuronal loss, glyosis, and accumulations of mutated Htt, all associated with different underlying channels in the pathogenesis of the disease, such as: excitotoxicity, energy deficit (ATP depletion), reduction in the synthesis and release of neurotrophic factors (BDNF and GDNF), and oxidative stress. Oxidative stress is involved in the pathogenesis of various neurodegenerative diseases, including Huntington's disease, and numerous studies have shown the existence of oxidative damage in the plasma and tissue of patients suffering from this disease.


CONCLUSIONS
Oxidative stress in patients with Huntington's disease may be used as an evolutionary-prognostic marker of both the disease and therapeutic effectiveness, as well as an interesting field of research for the development of new therapeutic strategies.
Tasset
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Departamento de Bioquímica y Biología Molecular, Facultad de Medicina, Universidad de Córdoba, Córdoba, España. b72tacui@uco.es
Sánchez
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F
Túnez
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D006801:Humans; D006816:Huntington Disease / Q000209:etiology / Q000378:metabolism*; D008967:Molecular Biology; D018384:Oxidative Stress