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多系统萎缩的疾病机制：意大利面的形状与多系统萎缩和帕金森病中涉及的α-突触核蛋白聚集体之间的相似之处能告诉我们什么。

Disease Mechanisms of Multiple System Atrophy: What a Parallel Between the Form of Pasta and the Alpha-Synuclein Assemblies Involved in MSA and PD Tells Us.

作者信息

Melki Ronald

机构信息

Institut Francois Jacob (MIRCen), CEA and Laboratory of Neurodegenerative Diseases, CNRS, 18 Route du Panorama, 92265, Fontenay-Aux-Roses, France.

出版信息

Cerebellum. 2024 Feb;23(1):13-21. doi: 10.1007/s12311-022-01417-0. Epub 2022 Jun 3.

DOI:10.1007/s12311-022-01417-0
PMID:35657577
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10864476/
Abstract

Intracellular deposits rich in aggregated alpha-synuclein that appear within the central nervous system are intimately associated to Parkinson's disease and multiple system atrophy. While it is understandable that the aggregation of proteins, which share no primary structure identity, such as alpha-synuclein and tau protein, leads to different diseases, that of a given protein yielding distinct pathologies is counterintuitive. This short review relates molecular and mechanistic processes to the observed pathological diversity associated to alpha-synuclein aggregation.

摘要

在中枢神经系统中出现的富含聚集性α-突触核蛋白的细胞内沉积物与帕金森病和多系统萎缩密切相关。虽然蛋白质聚集会导致不同疾病是可以理解的，比如没有一级结构同一性的α-突触核蛋白和tau蛋白，但特定蛋白质的聚集产生不同病理情况却有违直觉。这篇简短综述将分子和机制过程与观察到的与α-突触核蛋白聚集相关的病理多样性联系起来。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/772d/10864476/812f6c650ad4/12311_2022_1417_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/772d/10864476/dac765b09654/12311_2022_1417_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/772d/10864476/0b03024492e1/12311_2022_1417_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/772d/10864476/812f6c650ad4/12311_2022_1417_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/772d/10864476/dac765b09654/12311_2022_1417_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/772d/10864476/0b03024492e1/12311_2022_1417_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/772d/10864476/812f6c650ad4/12311_2022_1417_Fig3_HTML.jpg

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