% 0期刊文章%一个王Ningshan克里斯托弗•吉本斯% %画Kern %一个霍雷肖Kaufmann Jose-Alberto帕尔马% %罗伊·弗里曼% T皮肤多系统萎缩α-突触核蛋白沉积(S18.003) % D J神经病学2019% % P S18.003 % V 92% N 15补充% X目的:定义外围神经沉积MSA的α-突触核蛋白。首页背景:我们最近报道,α-synuclein皮肤自主神经纤维内可以发现帕金森病(PD)患者。我们现在报告磷酸化的沉积和总α-突触核蛋白在MSA患者,并比较这些结果与PD控制和个人。设计/方法:与MSA 29个人,22 PD患者和10个健康对照组临床检查,自主功能测试和皮肤活检是从多个近端和远端站点。皮肤活检PGP9.5染色,磷酸化和总α-synuclein比自主功能测试和结果。结果:MSA患者有证据的外围磷酸化α-synuclein存款在所有科目(P < 0.001与控制,没有发现α-synuclein)在近端和远端活检网站。显著差异-核蛋白MSA主题和PD主题之间沉积(P < 0.01)指出在所有活组织检查网站。结论:我们报告最大的皮肤研究α-核蛋白在个体与多个系统萎缩。与预期相反,周围沉积的磷酸化α-synuclein与MSA和PD出现在所有科目。这些发现可能提供见解的不同模式-核蛋白沉积在神经退行性疾病的范围。结果有重大影响疾病诊断、预后和治疗干预措施,改变疾病的自然史。披露:Gibbons博士已经收到个人赔偿咨询、担任科学顾问委员会说,或其他活动Lundbeck公司有限公司吉本斯博士已收到它是一家个人自主神经科学在一篇社论中补偿容量:基础和临床。 Dr. Gibbons has received research support from Grifols Inc. Dr. Wang has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Abide, Acetylon, Astellas, Biogen, Daiichi-Sankyo, Dong-A, Grace, Insys, Lundbeck, Nestle, Novartis, Pfizer, Vertex and Spinifex. Dr. Kern has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with AbbVie Pharmaceuticals, Merz Pharma and SAI-Med Partners, LLC. Dr. Palma has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Lundbeck and Biogen. Dr. Palma has received personal compensation in an editorial capacity for Clinical Autonomic Research. Dr. Kaufmann has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Lundbeck, Theravance and Biogen. Dr. Kaufmann has received personal compensation in an editorial capacity for Clinical Autonomic Research. Dr. Kaufmann has received research support from Theravance. Dr. Freeman has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Abide, Astellas, Aptinyx, Biogen, Chromocell, Dong-A, Ironwood, Lundbeck, MundiPharma, NeuroBo, Novartis, Pfizer, Regenacy, Toray and Theravance. Dr. Freeman has received personal compensation in an editorial capacity for Autonomic Neuroscience: Basic and Clinical. Dr. Freeman has received compensation for serving on the Board of Directors of NeuroBo. Dr. Freeman holds stock and/or stock options in NeuroBo. Dr. Freeman has received research support from Pfizer. %U