Division of Neuroscience, Department of Pharmacology, Smt. Kishoritai Bhoyar College of Pharmacy, New Kamptee, Nagpur, Maharashtra 441002, India.
Ageing Res Rev. 2024 Nov;101:102482. doi: 10.1016/j.arr.2024.102482. Epub 2024 Sep 3.
An autosomal dominant neurodegenerative disease called Huntington's disease (HD) is characterized by motor dysfunction, cognitive decline, and a variety of psychiatric symptoms due to the expansion of polyglutamine in the Huntingtin gene. The disease primarily affects the striatal neurons within the basal ganglia, leading to significant neuronal loss and associated symptoms such as chorea and dystonia. Current therapeutic approaches focus on symptom management without altering the disease's progression, highlighting a pressing need for novel treatment strategies. Recent studies have identified imidazoline receptors (IRs) as promising targets for neuroprotective and disease-modifying interventions in HD. IRs, particularly the I1 and I2 subtypes, are involved in critical physiological processes such as neurotransmission, neuronal excitability, and cell survival. Activation of these receptors has been shown to modulate neurotransmitter release and provide neuroprotective effects in preclinical models of neurodegeneration. This review discusses the potential of IR-targeted therapies to not only alleviate multiple symptoms of HD but also possibly slow the progression of the disease. We emphasize the necessity for ongoing research to further elucidate the role of IRs in HD and develop selective ligands that could lead to effective and safe treatments, thereby significantly improving patient outcomes and quality of life.
亨廷顿病(HD)是一种常染色体显性神经退行性疾病,其特征是由于亨廷顿基因中的多聚谷氨酰胺扩展,导致运动功能障碍、认知能力下降和多种精神症状。该疾病主要影响基底神经节中的纹状体神经元,导致显著的神经元丧失和相关症状,如舞蹈症和肌张力障碍。目前的治疗方法侧重于症状管理而不改变疾病的进展,这突出表明需要新的治疗策略。最近的研究表明,咪唑啉受体(IR)是 HD 中神经保护和疾病修饰干预的有前途的靶点。IR 特别是 I1 和 I2 亚型,参与关键的生理过程,如神经递质传递、神经元兴奋性和细胞存活。已经表明,这些受体的激活可以调节神经递质的释放,并在神经退行性变的临床前模型中提供神经保护作用。这篇综述讨论了针对 IR 的治疗方法不仅可以缓解 HD 的多种症状,而且还可能减缓疾病的进展。我们强调需要进行持续的研究,以进一步阐明 IR 在 HD 中的作用,并开发出选择性配体,从而可能导致有效和安全的治疗方法,从而显著改善患者的预后和生活质量。