Chen Alex Y, Xia Shouzhen, Wilburn Paul, Tully Tim
Dart Neuroscience LLC, San Diego, California, United States of America; Cold Spring Harbor Laboratory, Cold Spring Harbor, New York, United States of America; Graduate Program in Neuroscience, SUNY Stony Brook, Stony Brook, New York, United States of America.
Dart Neuroscience LLC, San Diego, California, United States of America.
PLoS One. 2014 May 30;9(5):e97758. doi: 10.1371/journal.pone.0097758. eCollection 2014.
Parkinson's disease (PD) is the most common motor neurodegenerative disorder. Olfactory dysfunction is a prevalent feature of PD. It often precedes motor symptoms by several years and is used in assisting PD diagnosis. However, the cellular and molecular bases of olfactory dysfunction in PD are not known. The fruit fly Drosophila melanogaster, expressing human alpha-synuclein protein or its mutant, A30P, captures several hallmarks of PD and has been successfully used to model PD in numerous studies. First, we report olfactory deficits in fly expressing A30P (A30P), showing deficits in two out of three olfactory modalities, tested--olfactory acuity and odor discrimination. The remaining third modality is odor identification/naming. Second, oxidative stress is an important environmental risk factor of PD. We show that oxidative stress exacerbated the two affected olfactory modalities in younger A30P flies. Third, different olfactory receptor neurons are activated differentially by different odors in flies. In a separate experiment, we show that the odor discrimination deficit in A30P flies is general and not restricted to a specific class of chemical structure. Lastly, by restricting A30P expression to dopamine, serotonin or olfactory receptor neurons, we show that A30P expression in dopamine neurons is necessary for development of both acuity and discrimination deficits, while serotonin and olfactory receptor neurons appeared not involved. Our data demonstrate olfactory deficits in a synuclein fly PD model for exploring olfactory pathology and physiology, and for monitoring PD progression and treatment.
帕金森病(PD)是最常见的运动性神经退行性疾病。嗅觉功能障碍是PD的一个普遍特征。它通常在运动症状出现前数年就已存在,并用于辅助PD的诊断。然而,PD中嗅觉功能障碍的细胞和分子基础尚不清楚。表达人α-突触核蛋白或其突变体A30P的果蝇黑腹果蝇体现了PD的几个特征,并已在众多研究中成功用于建立PD模型。首先,我们报告了表达A30P(A30P)的果蝇存在嗅觉缺陷,在测试的三种嗅觉模式中的两种——嗅觉敏锐度和气味辨别力方面表现出缺陷。剩下的第三种模式是气味识别/命名。其次,氧化应激是PD的一个重要环境风险因素。我们表明,氧化应激加剧了年轻A30P果蝇中受影响的两种嗅觉模式。第三,果蝇中不同的嗅觉受体神经元被不同的气味以不同方式激活。在另一个实验中,我们表明A30P果蝇的气味辨别缺陷是普遍存在的,并不局限于特定的化学结构类别。最后,通过将A30P的表达限制在多巴胺能神经元、5-羟色胺能神经元或嗅觉受体神经元中,我们发现多巴胺能神经元中A30P的表达对于敏锐度和辨别力缺陷的发展是必要的,而5-羟色胺能神经元和嗅觉受体神经元似乎未参与其中。我们的数据证明了在一个突触核蛋白果蝇PD模型中存在嗅觉缺陷,这对于探索嗅觉病理生理学以及监测PD的进展和治疗具有重要意义。