997 resultados para Charlot, Joseph-Auguste (1...-1874)
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Donateur : Daubrée, Auguste (1814-1896)
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Titre original : The ghost hunter and his family
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Machado-Joseph disease or spinocerebellar ataxia type 3, the most common dominantly-inherited spinocerebellar ataxia, results from translation of the polyglutamine-expanded and aggregation prone ataxin 3 protein. Clinical manifestations include cerebellar ataxia and pyramidal signs and there is no therapy to delay disease progression. Beclin 1, an autophagy-related protein and essential gene for cell survival, is decreased in several neurodegenerative disorders. This study aimed at evaluating if lentiviral-mediated beclin 1 overexpression would rescue motor and neuropathological impairments when administered to pre- and post-symptomatic lentiviral-based and transgenic mouse models of Machado-Joseph disease. Beclin 1-mediated significant improvements in motor coordination, balance and gait with beclin 1-treated mice equilibrating longer periods in the Rotarod and presenting longer and narrower footprints. Furthermore, in agreement with the improvements observed in motor function beclin 1 overexpression prevented neuronal dysfunction and neurodegeneration, decreasing formation of polyglutamine-expanded aggregates, preserving Purkinje cell arborization and immunoreactivity for neuronal markers. These data show that overexpression of beclin 1 in the mouse cerebellum is able to rescue and hinder the progression of motor deficits when administered to pre- and post-symptomatic stages of the disease.
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Référence bibliographique : Rol, 57665
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Référence bibliographique : Rol, 57604
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Référence bibliographique : Rol, 57687
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Référence bibliographique : Rol, 57726
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Référence bibliographique : Rol, 57691
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Référence bibliographique : Rol, 57603
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Référence bibliographique : Rol, 57602
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Référence bibliographique : Rol, 57601