Center for Movement Disorders, Department of Neurology , Beijing Tiantan Hospital, Capital Medical University , Beijing 100070 , China.
China National Clinical Research Center for Neurological Diseases , Beijing Tiantan Hospital, Capital Medical University , Beijing 100070 , China.
ACS Chem Neurosci. 2019 Feb 20;10(2):812-823. doi: 10.1021/acschemneuro.8b00383. Epub 2019 Feb 11.
Parkinson's disease (PD) is a common neurodegenerative disorder. To date, the diagnosis of PD relies mainly on clinical manifestations whereas neuropathological confirmation of the brain is only possible with postmortem studies. Neuronal loss in the substantia nigra pars compacta (SNc) associated with Lewy bodies/neurites is the pathological hallmark feature of PD. The major component of Lewy pathology (LP) is misfolded alpha-synuclein (α-SYN). There is evidence that the distribution of LP is not only limited to the brain but extends to peripheral tissues, including gastrointestinal tract, salivary glands, olfactory mucosa, skin, retina, adrenal gland, and heart. Sensitivity and specificity of α-SYN detection in PD vary greatly among studies due to methodological heterogeneity, such as sampling sites and size, tissue preparation, staining techniques, and antibodies used. Of note, α-SYN has also been found in preclinical and prodromal PD. Further in vivo studies focusing on favorable biopsy sites and standard techniques are needed to get better understanding of α-SYN deposits in preclinical, prodromal, and clinical PD.
帕金森病（PD）是一种常见的神经退行性疾病。迄今为止，PD 的诊断主要依赖于临床表现，而只有通过尸检研究才能对大脑进行神经病理学确认。与路易体/神经纤维相关的黑质致密部（SNc）神经元丧失是 PD 的病理标志特征。路易病理（LP）的主要成分是错误折叠的α-突触核蛋白（α-SYN）。有证据表明，LP 的分布不仅局限于大脑，还延伸到外周组织，包括胃肠道、唾液腺、嗅粘膜、皮肤、视网膜、肾上腺和心脏。由于方法学的异质性，如采样部位和大小、组织准备、染色技术和使用的抗体，α-SYN 在 PD 中的检测的敏感性和特异性在不同的研究中差异很大。值得注意的是，α-SYN 也在临床前和前驱期 PD 中被发现。需要进一步的体内研究，侧重于有利的活检部位和标准技术，以更好地了解临床前、前驱期和临床 PD 中的α-SYN 沉积。
