Department of Neurology, Complejo Hospitalario de Navarra Pamplona, Spain ; Instituto de Investigación Sanitaria de Navarra (IDISNA) Pamplona, Spain.
Instituto de Investigación Sanitaria de Navarra (IDISNA) Pamplona, Spain ; Brain Bank, Navarra Biomed Pamplona, Spain.
Front Neuroanat. 2015 Mar 11;9:25. doi: 10.3389/fnana.2015.00025. eCollection 2015.
To analyze the frequency and distribution of α-synuclein deposits in progressive supranuclear palsy (PSP).
The brains of 25 cases of pathologically confirmed PSP were evaluated with immunohistochemistry for α-synuclein and tau. Multiple immunofluorescent stains were applied to analyze the expression of tau and α-synuclein aggregates in catecholaminergic neurons. Patients' clinical symptoms were retrospectively recorded.
Deposits α-synuclein in the form of typical Lewy bodies (LBs) were only found in two PSP cases (8%) that fulfilled the clinical subtype of PSP known as Richardson's syndrome (RS). LBs were present in the locus ceruleus (LC), substantia nigra pars compacta (SNc), basal forebrain, amygdala and cingulated cortex in a distribution mimicking that of Parkinson's disease (PD). Triple-immunolabeling revealed co-expression of α-synuclein and tau proteins in some tyrosine hydroxilase (TH)-positive neurons of the LC and SNc.
There is no apparent clinical correlation between the presence of LBs in PSP. Tau protein co-aggregate with α-synuclein in catecholaminergic neurons of PSP brains suggesting a synergistic interaction between the two proteins. This is in keeping with the current view of neurodegenerative disorders as "misfolded protein diseases".
分析帕金森病相关核团路易小体（Lewy bodies，LB）在进行性核上性麻痹（progressive supranuclear palsy，PSP）中的分布频率。
对 25 例经病理证实的 PSP 患者的脑组织进行α-突触核蛋白和 tau 的免疫组织化学分析。应用多重免疫荧光染色分析儿茶酚胺能神经元中 tau 和α-突触核蛋白聚集物的表达。回顾性记录患者的临床症状。
只有在符合 Richardson 综合征（RS）临床亚型的 2 例 PSP 患者（8%）中发现了以典型路易小体（LB）形式存在的α-突触核蛋白沉积。LB 存在于蓝斑（LC）、黑质致密部（SNc）、基底前脑、杏仁核和扣带回皮质，分布类似于帕金森病（PD）。三重免疫标记显示 LC 和 SNc 中的一些酪氨酸羟化酶（TH）阳性神经元中存在α-突触核蛋白和 tau 蛋白的共表达。
在 PSP 中 LB 的存在与临床无明显相关性。tau 蛋白与 PSP 脑中的α-突触核蛋白共聚集，提示这两种蛋白之间存在协同作用。这与神经退行性疾病是“错误折叠蛋白疾病”的观点一致。
