Huntington's disease (HD) is a neurodegenerative disease characterized by prominent loss of neurons in the striatum and cortex. Traditionally research in HD has focused on brain changes as they cause progressive motor dysfunction, cognitive decline and psychiatric disorders. The discovery that huntingtin protein (HTT) and its mutated form (mHTT) are expressed not only in the brain but also in different organs and tissues paved the way for the hypothesis that HD might affect regions beyond the central nervous system (CNS). Besides pathological deposition of mHTT, other mechanisms, including inflammation, seem to underlie HD pathogenesis and progression. Altered inflammation can be evidenced even before the onset of classical symptoms of HD. Herein, we will discuss current pre-clinical and clinical evidence on immune/inflammatory changes in peripheral organs during HD development and progression. The understanding of the impact of inflammation on peripheral organs may open new venues for the development of novel therapeutic targets in HD.

中文翻译：

---

亨廷顿病中的炎症：旧故事的一些新转折

亨廷顿病 (HD) 是一种神经退行性疾病，其特征是纹状体和皮质中的神经元明显丢失。传统上 HD 的研究集中在大脑变化，因为它们会导致进行性运动功能障碍、认​​知能力下降和精神障碍。亨廷顿蛋白 (HTT) 及其突变形式 (mHTT) 不仅在大脑中而且在不同器官和组织中表达的发现为 HD 可能影响中枢神经系统 (CNS) 以外区域的假设铺平了道路。除了 mHTT 的病理沉积外，包括炎症在内的其他机制似乎是 HD 发病机制和进展的基础。甚至在 HD 的典型症状出现之前就可以证明炎症的改变。在此处，我们将讨论当前关于 HD 发展和进展期间外周器官免疫/炎症变化的临床前和临床证据。了解炎症对外周器官的影响可能为开发新的 HD 治疗靶点开辟新的途径。