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用于模拟帕金森病中α-突触核蛋白传播的患者来源诱导多能干细胞和类器官

Patient-Derived Induced Pluripotent Stem Cells and Organoids for Modeling Alpha Synuclein Propagation in Parkinson's Disease.

作者信息

Koh Yong Hui, Tan Li Yi, Ng Shi-Yan

机构信息

Institute of Molecular and Cell Biology, Singapore, Singapore.

Department of Physiology, Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore.

出版信息

Front Cell Neurosci. 2018 Nov 9;12:413. doi: 10.3389/fncel.2018.00413. eCollection 2018.

Abstract

Parkinson's disease (PD) is an age-associated, progressive neurodegenerative disorder characterized by motor impairment and in some cases cognitive decline. Central to the disease pathogenesis of PD is a small, presynaptic neuronal protein known as alpha synuclein (a-syn), which tends to accumulate and aggregate in PD brains as Lewy bodies or Lewy neurites. Numerous and studies confirm that a-syn aggregates can be propagated from diseased to healthy cells, and it has been suggested that preventing the spread of pathogenic a-syn species can slow PD progression. In this review, we summarize the works of recent literature elucidating mechanisms of a-syn propagation, and discussed the advantages in using patient-derived induced pluripotent stem cells (iPSCs) and/or induced neurons to study a-syn transmission.

摘要

帕金森病(PD)是一种与年龄相关的进行性神经退行性疾病,其特征为运动障碍,在某些情况下还伴有认知衰退。PD发病机制的核心是一种称为α-突触核蛋白(α-syn)的小的突触前神经元蛋白,它在PD患者大脑中倾向于聚积并形成路易小体或路易神经突。大量研究证实,α-syn聚集体可从病变细胞传播至健康细胞,并且有人提出,阻止致病性α-syn物种的传播可减缓PD的进展。在本综述中,我们总结了近期文献中阐明α-syn传播机制的研究工作,并讨论了使用患者来源的诱导多能干细胞(iPSC)和/或诱导神经元来研究α-syn传播的优势。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ba53/6240766/d5af5632f854/fncel-12-00413-g0001.jpg

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