The approval of istradefylline, an adenosine 2A receptor (A2AR) antagonist, as an addon treatment in adult patients with Parkinson's disease by the Food and Drug Administration (FDA) and European Medicines Agency (EMA), is the latest proof of the importance of the adenosinergic system in the nervous system. Adenosine is an endogenous purine nucleoside with a role as a modulator of both neurotransmission and the inflammatory response. As such, the expression pattern of the 4 adenosine receptors (A1R, A2AR, A2BR and A3R) and the extracellular adenosine levels have attracted great interest in the pathogenesis and possible treatment of rare neurodegenerative diseases with motor symptoms. These include Huntington's Disease (HD), Amyotrophic Lateral Sclerosis (ALS), Multiple Sclerosis (MS), Restless Legs Syndrome (RLS) and Machado-Joseph Disease (MJD, also known as spinocerebellar ataxia type 3, SCA3). In this review, we shall focus on the role of the different adenosine receptor subtypes in the development and possible treatment of the aforementioned rare neurodegenerative diseases with motor symptoms using the currently available data. The last section discusses the possibility of a role for the adenosine receptors in the treatment of other rare diseases based on the available molecular pathology knowledge.

中文翻译：

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腺苷受体在具有运动症状的罕见神经退行性疾病中的作用。


美国食品药品监督管理局 (FDA) 和欧洲药品管理局 (EMA) 批准伊曲茶碱（一种腺苷 2A 受体 (A2AR) 拮抗剂）作为成人帕金森病患者的附加治疗药物，这是该药物重要性的最新证明。神经系统中的腺苷能系统。腺苷是一种内源性嘌呤核苷，具有神经传递和炎症反应调节剂的作用。因此，4 种腺苷受体（A1R、A2AR、A2BR 和 A3R）的表达模式和细胞外腺苷水平引起了人们对具有运动症状的罕见神经退行性疾病的发病机制和可能治疗的极大兴趣。这些疾病包括亨廷顿病 (HD)、肌萎缩侧索硬化症 (ALS)、多发性硬化症 (MS)、不宁腿综合征 (RLS) 和马查多-约瑟夫病 (MJD，也称为 3 型脊髓小脑共济失调，SCA3)。在这篇综述中，我们将利用当前可用的数据，重点关注不同腺苷受体亚型在上述具有运动症状的罕见神经退行性疾病的发展和可能治疗中的作用。最后一节根据现有的分子病理学知识讨论了腺苷受体在治疗其他罕见疾病中发挥作用的可能性。