Risby-Jones Grace, Lee John D, Woodruff Trent M, Fung Jenny N
School of Biomedical Sciences, The University of Queensland, St Lucia, QLD, Australia.
Queensland Brain Institute, The University of Queensland, St Lucia, QLD, Australia.
Front Neurol. 2024 Jun 10;15:1384480. doi: 10.3389/fneur.2024.1384480. eCollection 2024.
Huntington's disease (HD) is a debilitating neurodegenerative condition characterized by motor, cognitive and psychiatric abnormalities. Immune dysregulation, prominently featuring increased immune activity, plays a significant role in HD pathogenesis. In addition to the central nervous system (CNS), systemic innate immune activation and inflammation are observed in HD patients, exacerbating the effects of the Huntingtin (HTT) gene mutation. Recent attention to sex differences in HD symptom severity underscores the need to consider gender as a biological variable in neurodegenerative disease research. Understanding sex-specific immune responses holds promise for elucidating HD pathophysiology and informing targeted treatment strategies to mitigate cognitive and functional decline. This perspective will highlight the importance of investigating gender influence in HD, particularly focusing on sex-specific immune responses predisposing individuals to disease.
亨廷顿病(HD)是一种使人衰弱的神经退行性疾病,其特征为运动、认知和精神异常。免疫失调,突出表现为免疫活性增加,在HD发病机制中起重要作用。除中枢神经系统(CNS)外,HD患者还存在全身先天性免疫激活和炎症,这加剧了亨廷顿蛋白(HTT)基因突变的影响。最近对HD症状严重程度性别差异的关注强调了在神经退行性疾病研究中需要将性别视为一个生物学变量。了解性别特异性免疫反应有望阐明HD的病理生理学,并为减轻认知和功能衰退的靶向治疗策略提供依据。这一观点将强调研究性别对HD影响的重要性,特别是关注使个体易患该病的性别特异性免疫反应。