Parkinson's disease (PD) is a major neurodegenerative disorder characterized by a variety of non-motor symptoms in addition to the well-recognized motor dysfunctions that have commanded primary interest. We previously described a new PD mouse model based on heterozygous disruption of the B4galnt1 gene leading to partial deficiency of the GM1 family of gangliosides that manifested several nigrostriatal neuropathological features of PD as well as movement impairment. We now show this mouse also suffers three non-motor symptoms characteristic of PD involving the gastrointestinal, sympathetic cardiac, and cerebral cognitive systems. Treatment of these animals with a synthetic form of GM1 ganglioside, produced by transfected E. coli, proved ameliorative of these symptoms as well as the motor defect. These findings further suggest subnormal GM1 to be a systemic defect constituting a major risk factor in sporadic PD and indicate the B4galnt1(+/-) (HT) mouse to be a true neuropathological model that recapitulates both motor and non-motor lesions of this condition.

中文翻译：

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缺乏GM1的小鼠表现出帕金森氏病的运动和非运动症状。合成GM1神经节苷脂治疗成功。

帕金森氏病（PD）是一种主要的神经退行性疾病，其特征是除了引起人们广泛关注的公认的运动功能障碍之外，还存在多种非运动症状。我们之前描述了一种新的PD小鼠模型，该模型基于B4galnt1基因的杂合破坏导致神经节苷脂GM1家族的部分缺失，表现出PD的几种黑纹状体神经病理学特征以及运动障碍。现在，我们显示该小鼠还患有PD的三个非运动症状，涉及胃肠道，交感性心脏和大脑认知系统。用转染的大肠杆菌生产的合成形式的GM1神经节苷脂治疗这些动物，证明可以改善这些症状以及运动缺陷。