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细胞朊蛋白在神经元中α-突触核蛋白运输中的作用。

Involvement of Cellular Prion Protein in α-Synuclein Transport in Neurons.

机构信息

Molecular and Cellular Neurobiotechnology, Institute of Bioengineering of Catalonia (IBEC), Parc Científic de Barcelona, Baldiri Reixac 15-21, E-08028, Barcelona, Spain.

Department of Cell Biology, Physiology and Immunology, Universitat de Barcelona, Barcelona, Spain.

出版信息

Mol Neurobiol. 2018 Mar;55(3):1847-1860. doi: 10.1007/s12035-017-0451-4. Epub 2017 Feb 22.

Abstract

The cellular prion protein, encoded by the gene Prnp, has been reported to be a receptor of β-amyloid. Their interaction is mandatory for neurotoxic effects of β-amyloid oligomers. In this study, we aimed to explore whether the cellular prion protein participates in the spreading of α-synuclein. Results demonstrate that Prnp expression is not mandatory for α-synuclein spreading. However, although the pathological spreading of α-synuclein can take place in the absence of Prnp, α-synuclein expanded faster in PrP-overexpressing mice. In addition, α-synuclein binds strongly on PrP-expressing cells, suggesting a role in modulating the effect of α-synuclein fibrils.

摘要

细胞朊病毒蛋白由 Prnp 基因编码,据报道它是β-淀粉样蛋白的受体。它们的相互作用是β-淀粉样蛋白寡聚物产生神经毒性作用的必要条件。在这项研究中,我们旨在探索细胞朊病毒蛋白是否参与α-突触核蛋白的传播。结果表明,细胞朊病毒蛋白的表达对于α-突触核蛋白的传播不是必需的。然而,尽管α-突触核蛋白的病理性传播可以在没有 Prnp 的情况下发生,但在 PrP 过表达的小鼠中,α-突触核蛋白的传播速度更快。此外,α-突触核蛋白在表达 PrP 的细胞上强烈结合,提示其在调节α-突触核蛋白纤维的作用中发挥作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6201/5840251/6858233d504d/12035_2017_451_Fig1_HTML.jpg

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