Neuropath Core

核心摘要 神经病理学核心C 约翰霍普金斯尤德尔帕金森病研究中心的神经病理学核心（核心C） 三大目标。第一个是对受试者进行死后神经病理学评估， 临床核心和分配尸检脑组织的研究项目1，2和4和蛋白质组学 核心第二个是获得所有死后组织的综合遗传图谱， 尤德尔中心收藏。三是为遗传学形态评价提供支持 工程小鼠模型的研究人员在项目1，2和4。核心C的具体目标如下： (1)安排和执行帕金森病（PD）和路易体病例的神经病理学尸检 疾病（LBD）和对照受试者，然后是临床核心，并制定病理诊断; (2)登记，准备，目录，并协助分析人类死后组织的情况下， PD/LBD和相关疾病，以及年龄匹配和年轻对照研究， 项目1、2和4以及蛋白质组学核心;（3）表征PD/LBD的分子遗传学谱 通过与NIA神经遗传学实验室的合作， （A. Singleton，Ph.D.）;（4）采用显微组织学技术制备小鼠脑组织， 项目1、2及4所界定的研究。 尽管最近在神经影像学方面取得了进展，但死后脑检查仍然是必不可少的。用于精确 在我们的经验中，PD（帕金森病）和LBD（路易体病）的诊断，约20%的病例 临床诊断为PD的患者有其他病因，并且>30%的病例有并存病。 此外，由于尸体解剖是研究分子/生化异常的组织的基本来源， 与PD/LBD相关的人脑，彻底的病理特征是重要的， 组织可用于项目1、2和4以及蛋白质组学核心提出的研究。核心C正在扩张 其死后组织收集包括大量来自年轻受试者（16至65岁）的标本 适用于检查PD/LBD病理学的非常早期阶段及其发病机制。这些组织 通过与约翰霍普金斯的Lieber发育障碍研究所合作加入。 最后，通过使用CoreC实现的最先进的形态学技术， 项目1、2和4将能够生产整个小鼠大脑的半透明制剂， 不仅询问单个神经元群体的分子事件，而且询问与PD相关的网络， LBD。

CORE SUMMARY Neuropathology Core C The Neuropathology Core (Core C) of the Johns Hopkins Udall Parkinson's Disease Research Center has three overarching goals. The first is to conduct postmortem neuropathological assessments in subjects from the Clinical Core and to distribute autopsy brain tissues for research to Projects 1, 2 & 4 and the Proteomic Core. The second is to obtain a comprehensive genetic profile of all the postmortem tissues available in the Udall Center collection. The third is to provide support for the morphological evaluation of genetically engineered mouse models by investigators in Projects 1, 2 & 4. The specific aims of Core C are as follows: (1) to arrange and perform neuropathological autopsies of cases of Parkinson's disease (PD) and Lewy body disease (LBD), and control subjects followed by the Clinical Core, and to formulate pathological diagnoses; (2) to accession, prepare, catalog, and assist in the analysis of human postmortem tissues from cases of PD/LBD and related disorders, as well as age-matched and also younger controls for studies proposed in Projects 1, 2 & 4 and the Proteomic Core; (3) to characterize the molecular genetic profiles of PD/LBD postmortem tissues available in the BRC through a collaboration with the Laboratory of Neurogenetics at NIA (A. Singleton, Ph.D.); and (4) to use CLARITY technology for preparation of mouse brain tissues in support of studies delineated in Projects 1, 2 & 4. Despite recent advances in neuroimaging postmortem brain examination remains indispensable. For accurate diagnosis of PD (Parkinson's disease) and LBD (Lewy body disease) in our experience, ~20% of cases clinically diagnosed as PD have other etiologies, and >30% of the cases have coexisting morbidities. Moreover, since autopsies are the essential source of tissues for studying molecular/biochemical abnormalities of the human brain associated with PD/LBD, a thorough pathological characterization is important before tissues can be used in studies proposed in Projects 1, 2 & 4 and by the Proteomic Core. Core C is expanding its postmortem tissue collection to include a large number of specimens from younger subjects (16 to 65 years) suitable to examine the very early stages of PD/LBD pathology and its pathogenesis. These tissues are accessioned through collaboration with the Lieber Institute for Developmental Disorders at Johns Hopkins. Finally, by using CLARITY, a state-of-the-art morphological technique implemented by Core C, investigators in Projects 1, 2 & 4 will be able to produce translucent preparations of whole mouse brains that allow interrogation of molecular events not only of single neuronal populations but of networks relevant to PD and LBD.