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由于野生型人源α-突触核蛋白水平升高导致的小鼠多巴胺能神经元丧失仅在加速衰老的条件下发生。

Dopaminergic neuron loss in mice due to increased levels of wild-type human α-Synuclein only takes place under conditions of accelerated aging.

机构信息

Departamento de Biología Celular, Biología Funcional y Antropología Física, Universitat de València, Valencia, Spain.

Instituto de Biotecnología y Biomedicina (BioTecMed), Universitat de València, Valencia, Spain.

出版信息

Sci Rep. 2024 Jan 30;14(1):2490. doi: 10.1038/s41598-024-53093-1.

DOI:10.1038/s41598-024-53093-1
PMID:38291230
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10828501/
Abstract

Understanding the intricate pathogenic mechanisms behind Parkinson's disease (PD) and its multifactorial nature presents a significant challenge in disease modeling. To address this, we explore genetic models that better capture the disease's complexity. Given that aging is the primary risk factor for PD, this study investigates the impact of aging in conjunction with overexpression of wild-type human α-synuclein (α-Syn) in the dopaminergic system. This is achieved by introducing a novel transgenic mouse strain overexpressing α-Syn under the TH-promoter within the senescence-accelerated SAMP8 (P8) genetic background. Behavioral assessments, conducted at both 10 and 16 months of age, unveil motor impairments exclusive to P8 α-SynTg mice, a phenomenon conspicuously absent in α-SynTg mice. These findings suggest a synergistic interplay between heightened α-Syn levels and the aging process, resulting in motor deficits. These motor disturbances correlate with reduced dopamine (DA) levels, increased DA turnover, synaptic terminal loss, and notably, the depletion of dopaminergic neurons in the substantia nigra and noradrenergic neurons in the locus coeruleus. Furthermore, P8 α-SynTg mice exhibit alterations in gut transit time, mirroring early PD symptoms. In summary, P8 α-SynTg mice effectively replicate parkinsonian phenotypes by combining α-Syn transgene expression with accelerated aging. This model offers valuable insights into the understanding of PD and serves as a valuable platform for further research.

摘要

了解帕金森病(PD)背后复杂的发病机制及其多因素性质是疾病建模的一个重大挑战。为了解决这个问题,我们探索了更好地捕捉疾病复杂性的遗传模型。鉴于衰老时 PD 的主要风险因素,本研究调查了衰老与多巴胺能系统中野生型人α-突触核蛋白(α-Syn)过表达的共同作用对疾病的影响。这是通过在衰老加速 SAMP8(P8)遗传背景下,在 TH 启动子的控制下,引入一种新型过表达 α-Syn 的转基因小鼠品系来实现的。在 10 个月和 16 个月大时进行的行为评估揭示了仅在 P8 α-SynTg 小鼠中出现的运动障碍,而在 α-SynTg 小鼠中则没有这种现象。这些发现表明,α-Syn 水平升高与衰老过程之间存在协同作用,导致运动缺陷。这些运动障碍与多巴胺(DA)水平降低、DA 周转率增加、突触末梢丧失有关,值得注意的是,黑质中的多巴胺能神经元和蓝斑核中的去甲肾上腺素能神经元耗竭。此外,P8 α-SynTg 小鼠表现出肠道转运时间的改变,这与早期 PD 症状相似。总之,P8 α-SynTg 小鼠通过将 α-Syn 转基因表达与加速衰老相结合,有效地复制了帕金森病的表型。该模型为理解 PD 提供了有价值的见解,并为进一步的研究提供了一个有价值的平台。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0bce/10828501/29989faddfaa/41598_2024_53093_Fig5_HTML.jpg
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