The autophagy-enhancing drug carbamazepine improves neuropathology and motor impairment in mouse models of Machado–Joseph disease,Neuropathology and Applied Neurobiology

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The autophagy-enhancing drug carbamazepine improves neuropathology and motor impairment in mouse models of Machado–Joseph disease
Neuropathology and Applied Neurobiology ( IF 4.0 ) Pub Date : 2021-08-25 , DOI: 10.1111/nan.12763
Ana Vasconcelos-Ferreira _{1,

2,

3} , Sara Carmo-Silva _{1,

2} , José Miguel Codêsso _{1,

2} , Patrick Silva _{1,

2,

3} , Alberto Rolim Muro Martinez ₄ , Marcondes Cavalcante França ₄ , Clévio Nóbrega _{1,

2} , Luís Pereira de Almeida _{1,

2,

3}

Affiliation

Machado–Joseph disease (MJD), or spinocerebellar ataxia type 3 (SCA3), is the most common autosomal dominantly-inherited ataxia worldwide and is characterised by the accumulation of mutant ataxin-3 (mutATXN3) in different brain regions, leading to neurodegeneration. Currently, there are no available treatments able to block disease progression. In this study, we investigated whether carbamazepine (CBZ) would activate autophagy and mitigate MJD pathology.

中文翻译：

自噬增强药物卡马西平可改善马查多-约瑟夫病小鼠模型的神经病理学和运动障碍

Machado-Joseph 病 (MJD) 或脊髓小脑性共济失调 3 型 (SCA3) 是世界范围内最常见的常染色体显性遗传性共济失调，其特征是突变型ataxin-3 (mutATXN3) 在不同脑区的积累，导致神经退行性变。目前，没有可用的治疗方法能够阻止疾病进展。在这项研究中，我们研究了卡马西平 (CBZ) 是否会激活自噬并减轻 MJD 病理学。

更新日期：2021-08-25

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