帕金森病患者血液中抗聚集的α-突触核蛋白四聚体减少。

IF 9 1区 医学 Q1 MEDICINE, RESEARCH & EXPERIMENTAL
EMBO Molecular Medicine Pub Date : 2024-07-01 Epub Date: 2024-06-05 DOI:10.1038/s44321-024-00083-5
Laura de Boni, Amber Wallis, Aurelia Hays Watson, Alejandro Ruiz-Riquelme, Louise-Ann Leyland, Thomas Bourinaris, Naomi Hannaway, Ullrich Wüllner, Oliver Peters, Josef Priller, Björn H Falkenburger, Jens Wiltfang, Mathias Bähr, Inga Zerr, Katharina Bürger, Robert Perneczky, Stefan Teipel, Matthias Löhle, Wiebke Hermann, Björn-Hendrik Schott, Kathrin Brockmann, Annika Spottke, Katrin Haustein, Peter Breuer, Henry Houlden, Rimona S Weil, Tim Bartels
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引用次数: 0

摘要

α-突触核蛋白蛋白在神经元、神经胶质细胞和其他组织中的积累和聚集决定了帕金森病(PD)等突触核蛋白病。我们以前曾证明,α-突触核蛋白四聚体的不稳定性与因 SNCA 突变导致的家族性帕金森病有关,并根据经典的布拉克扩散理论证明了散发性帕金森病患者中α-突触核蛋白多聚体的脑区特异性改变。在本研究中,我们评估了G51D突变的家族性帕金森病患者和散发性帕金森病患者血液中细胞膜α-突触核蛋白无序多聚体和高序多聚体的相对水平。我们采用了一种适用于人体 EDTA 全血的体外交联方案。与对照组相比,家族性帕金森病和散发性帕金森病患者血液中高阶α-突触核蛋白四聚体的相对水平降低了。有趣的是,在无症状的G51D携带者中,α-突触核蛋白四聚体的相对数量已经减少,这支持了α-突触核蛋白多聚体失稳先于临床帕金森病发生的假设。因此,我们的数据表明,测量血液中的α-突触核蛋白四聚体有可能成为一种简便的生物标记检测方法,用于早期检测和定量跟踪帕金森病的进展。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Aggregation-resistant alpha-synuclein tetramers are reduced in the blood of Parkinson's patients.

Synucleinopathies such as Parkinson's disease (PD) are defined by the accumulation and aggregation of the α-synuclein protein in neurons, glia and other tissues. We have previously shown that destabilization of α-synuclein tetramers is associated with familial PD due to SNCA mutations and demonstrated brain-region specific alterations of α-synuclein multimers in sporadic PD patients following the classical Braak spreading theory. In this study, we assessed relative levels of disordered and higher-ordered multimeric forms of cytosolic α-synuclein in blood from familial PD with G51D mutations and sporadic PD patients. We used an adapted in vitro-cross-linking protocol for human EDTA-whole blood. The relative levels of higher-ordered α-synuclein tetramers were diminished in blood from familial PD and sporadic PD patients compared to controls. Interestingly, the relative amount of α-synuclein tetramers was already decreased in asymptomatic G51D carriers, supporting the hypothesis that α-synuclein multimer destabilization precedes the development of clinical PD. Our data, therefore suggest that measuring α-synuclein tetramers in blood may have potential as a facile biomarker assay for early detection and quantitative tracking of PD progression.

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来源期刊
EMBO Molecular Medicine
EMBO Molecular Medicine 医学-医学:研究与实验
CiteScore
17.70
自引率
0.90%
发文量
105
审稿时长
4-8 weeks
期刊介绍: EMBO Molecular Medicine is an open access journal in the field of experimental medicine, dedicated to science at the interface between clinical research and basic life sciences. In addition to human data, we welcome original studies performed in cells and/or animals provided they demonstrate human disease relevance. To enhance and better specify our commitment to precision medicine, we have expanded the scope of EMM and call for contributions in the following fields: Environmental health and medicine, in particular studies in the field of environmental medicine in its functional and mechanistic aspects (exposome studies, toxicology, biomarkers, modeling, and intervention). Clinical studies and case reports - Human clinical studies providing decisive clues how to control a given disease (epidemiological, pathophysiological, therapeutic, and vaccine studies). Case reports supporting hypothesis-driven research on the disease. Biomedical technologies - Studies that present innovative materials, tools, devices, and technologies with direct translational potential and applicability (imaging technologies, drug delivery systems, tissue engineering, and AI)
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