Cerebrospinal fluid from patients with multiple system atrophy promotes in vitro α-synuclein fibril formation
Cerebrospinal fluid from patients with multiple system atrophy promotes in vitro α-synuclein fibril formation
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DOI：
10.1016/j.neulet.2011.01.005
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发表时间：
2011-03-10
影响因子：
2.5
通讯作者：
Yamada, Masahito
中科院分区：
文献类型：
--
作者：
Hirohata, Mie;Ono, Kenjiro;Yamada, Masahito
中枢神经系统（CNS）中α-突触核蛋白（alpha S）的聚集是多系统萎缩（MSA）和路易体疾病（包括帕金森病（PD）和路易体痴呆症（DLB）（α-突触核蛋白病））的标志。为了检验α-突触核蛋白病患者具有有利于α S聚集的CNS环境这一假设，我们检测了来自MSA（n = 20）、DLB（n = 8）和PD（n = 10）患者的脑脊液（CSF）对体外aS原纤维的影响。在pH7.5和37 ℃下，使用噻吩硫菌素S的荧光光谱法，与遗传性脊髓小脑共济失调（hSCA）（n = 16）的患者相比，紧张型头痛7例。来自MSA患者的CSF（MSA-CSF）比PD-、hSCA-或头痛-CSF更强烈地促进faS形成。电镜分析表明，MSA-CSF中形成的faS的宽度明显大于其他细胞。MSA可能具有特别有利于f α S形成的CSF环境。(C)2011爱思唯尔爱尔兰有限公司保留所有权利。
The aggregation of alpha-synuclein (alpha S) in the central nervous system (CNS) is the hallmark of multiple system atrophy (MSA) and Lewy body diseases including Parkinson's disease (PD) and dementia with Lewy bodies (DLB) (alpha-synucleinopathies). To test the hypothesis that patients with alpha-synucleinopathies have a CNS environment favorable for alpha S aggregation, we examined the influence of cerebrospinal fluid (CSF) from patients with MSA (n = 20), DLB (n = 8), and PD (n = 10) on in vitro aS fibril (f alpha S) formation at pH 7.5 and 37 degrees C using fluorescence spectroscopy with thiofiavin S, compared with those with hereditary spinocerebellar ataxia (hSCA) (n = 16), and tension-type headache (n = 7). CSF from MSA patients (MSA-CSF) promoted faS formation more strongly than PD-, hSCA-, or headache-CSF. By electron microscopic analyses, the width of faS formed in MSA-CSF was significantly greater than others. MSA may have a CSF environment particularly favorable for f alpha S formation. (C) 2011 Elsevier Ireland Ltd. All rights reserved.