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外周血单个核细胞中 GM1 水平低下:帕金森病早期诊断的希望?

Subnormal GM1 in PBMCs: Promise for Early Diagnosis of Parkinson's Disease?

机构信息

Department of Pharmacology, Physiology, and Neuroscience, Rutgers, The State University of NJ, Newark, NJ 07103, USA.

Department of Neurology, Columbia University Irving Medical Center, New York, NY 10032, USA.

出版信息

Int J Mol Sci. 2021 Oct 26;22(21):11522. doi: 10.3390/ijms222111522.

Abstract

The fact that Parkinson's disease (PD) pathologies are well advanced in most PD patients by the time of clinical elucidation attests to the importance of early diagnosis. Our attempt to achieve this has capitalized on our previous finding that GM1 ganglioside is expressed at subnormal levels in virtually all tissues of sporadic PD (sPD) patients including blood cells. GM1 is present in most vertebrate cells, is especially abundant in neurons where it was shown essential for their effective functioning and long term viability. We have utilized peripheral blood mononuclear cells (PBMCs) which, despite their low GM1, we found to be significantly lower in sPD patients compared to age-matched healthy controls. To quantify GM1 (and GD1a) we used high performance thin-layer chromatography combined with cholera toxin B linked to horseradish peroxidase, followed by densitometric quantification. GM1 was also deficient in PBMCs from PD patients with mutations in the glucocerebrosidase gene (PD-GBA), apparently even lower than in sPD. Reasons are given why we believe these results obtained with patients manifesting fully developed PD will apply as well to PD patients in preclinical stages-a topic for future study. We also suggest that these findings point to a potential disease altering therapy for PD once the early diagnosis is established.

摘要

帕金森病(PD)的病理学在大多数 PD 患者被临床阐明时已经相当严重,这一事实证明了早期诊断的重要性。我们试图实现这一目标的方法是基于我们之前的发现,即 GM1 神经节苷脂在包括血细胞在内的几乎所有散发性 PD(sPD)患者的组织中表达水平低于正常。GM1 存在于大多数脊椎动物细胞中,在神经元中尤为丰富,研究表明 GM1 对神经元的有效功能和长期存活至关重要。我们利用外周血单核细胞(PBMC),尽管其 GM1 水平较低,但与年龄匹配的健康对照组相比,我们发现 sPD 患者的 PBMC 明显更低。为了定量 GM1(和 GD1a),我们使用了高效薄层色谱法,结合霍乱毒素 B 与辣根过氧化物酶相连,然后进行密度定量分析。PD 患者的 GM1 也存在缺陷,这些患者的葡萄糖脑苷脂酶基因(PD-GBA)发生突变,显然比 sPD 患者还要低。我们给出了为什么我们认为这些在表现出完全发展的 PD 的患者中获得的结果也将适用于临床前阶段的 PD 患者的原因——这是未来研究的一个主题。我们还提出,一旦早期诊断确立,这些发现将为 PD 患者提供一种潜在的改变疾病的治疗方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b427/8583888/960a5667297b/ijms-22-11522-g001.jpg

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