Distinctive distribution of phospho-alpha-synuclein in dermal nerves in multiple system atrophy
Distinctive distribution of phospho-alpha-synuclein in dermal nerves in multiple system atrophy
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DOI：
10.1002/mds.26293
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发表时间：
2015-10-01
影响因子：
8.6
通讯作者：
Sommer, Claudia
中科院分区：
文献类型：
--
作者：
Doppler, Kathrin;Weis, Jessica;Sommer, Claudia
背景MSA的特征是在少突胶质细胞和中枢神经系统(CNS)神经元中沉积α-突触核蛋白(-Syn)。在最近检测了帕金森病(PD)患者真皮神经纤维中的磷酸化Syn(p-Syn)后，我们对MSA患者的皮肤活检进行了评估，以评估其作为生物标志物的潜在作用。方法对12例MSA患者、30例特发性PD患者、15例正常对照的皮肤活检组织进行分析。结果67%的MSA患者和帕金森病患者的真皮神经中存在P-Syn，而在连续15个切片分析中，肌萎缩侧索硬化症患者和正常对照组均未发现P-Syn。通过对连续切片的分析，灵敏度可分别提高到75%和73%。PD组p-Syn主要分布在自主神经纤维中，而MSA组则主要分布于无髓鞘的躯体感觉纤维中。结论MSA的Syn病变不局限于中枢神经系统，皮肤活检可能有助于p-Syn的生前研究。(C)2015年国际帕金森病和运动障碍协会
BackgroundMSA is characterized by deposition of alpha-synuclein (-Syn) in oligodendrocytes and central nervous system (CNS) neurons. After recently detecting phospho--Syn (p--Syn) in dermal nerve fibers of patients with Parkinson's disease (PD), we assessed skin biopsies from patients with MSA to evaluate its potential role as a biomarker.MethodsSkin biopsies of patients with MSA (n=12), idiopathic PD (n=30), tauopathies (n=15), and normal controls (n=39) were analyzed. P--Syn within dermal nerves was detected by immunofluorescence staining.Resultsp--Syn was found in 67% of patients with MSA and Parkinson's disease, but not in patients with tauopathy or controls when analyzing 15 consecutive sections. Sensitivity could be increased to 75% and 73%, respectively, by analyzing serial sections. In contrast to PD, where p--Syn clustered in autonomic fibers, deposits were mainly found in unmyelinated somatosensory fibers in MSA.Conclusion-Syn pathology in MSA is not restricted to the CNS, and skin biopsy may be useful for the premortem study of p--Syn. (c) 2015 International Parkinson and Movement Disorder Society