There is currently no disease-modifying treatment for Huntington’s disease (HD), which is characterized by chorea motor impairment and cognitive decline. The zinc ionophore, PBT2, was previously shown to improve the phenotype of a HD mouse model and reported efficacy in certain cognitive tests in a phase II clinical trial in HD. Here we report that zinc deficiency is a feature of the hippocampus and cortex in the R6/1 mouse model of HD. Low cortical zinc has been shown to induce cognitive impairment, and indeed, dietary restriction of zinc in R6/1 mice was associated with cognitive impairment in the Y-maze, an exacerbated hippocampal long-term potentiation (LTP) deficit and reduction of AMPA receptors (and not other glutamatergic receptors). These data reveal the importance of zinc in maintaining brain function in HD.

中文翻译：

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脑缺锌会加剧亨廷顿病 R6/1 模型的认知衰退。

目前还没有针对亨廷顿病 (HD) 的疾病缓解治疗，其特征是舞蹈病运动障碍和认知能力下降。锌离子载体 PBT2 先前被证明可以改善 HD 小鼠模型的表型，并在 HD 的 II 期临床试验中报告了某些认知测试的功效。在这里，我们报告缺锌是 HD 的 R6/1 小鼠模型中海马和皮质的一个特征。低皮质锌已被证明会诱发认知障碍，事实上，R6/1 小鼠中锌的饮食限制与 Y 迷宫中的认知障碍、海马长期增强 (LTP) 缺陷加剧和 AMPA 受体减少有关（而不是其他谷氨酸能受体）。这些数据揭示了锌在维持 HD 脑功能中的重要性。