肌肉特异性受体酪氨酸激酶（MuSK）重症肌无力。
Muscle-Specific Receptor Tyrosine Kinase (MuSK) Myasthenia Gravis.
机构信息
Department of Neurology, University of South Florida Morsani College of Medicine, 2 Tampa General Circle 6th Floor, Room 6091, Tampa, FL, 33606, USA.
Department of Neurology, University of South Florida Morsani College of Medicine, 12901 Bruce B. Downs Blvd MDC 55, Tampa, FL, 33612, USA.
出版信息
Curr Neurol Neurosci Rep. 2016 Jul;16(7):61. doi: 10.1007/s11910-016-0668-z.
Autoimmune myasthenia gravis (MG) is the prototypic, antibody-mediated neuromuscular disease and is characterized by a decrease in the number of functional acetylcholine receptors (AChR) within the muscle end plate zone of the neuromuscular junction (NMJ). Although the pathophysiology of AChR-mediated myasthenia gravis has been extensively studied over the last 40 years since its original description by Patrick and Lindstrom (Science 180:871-872, 1973), less is known about the much more recently described muscle-specific kinase (MuSK) antibody-mediated MG. MuSK-MG has features clinically distinct from Ach-R MG, as well as a different pattern of response to treatment and a unique immunopathogenesis.
自身免疫性重症肌无力 (MG) 是一种典型的抗体介导的神经肌肉疾病，其特征是在神经肌肉接头 (NMJ) 的肌肉终板区域内功能性乙酰胆碱受体 (AChR) 的数量减少。尽管自 Patrick 和 Lindstrom（Science 180:871-872, 1973）最初描述以来，在过去的 40 年中已经对 AChR 介导的重症肌无力的病理生理学进行了广泛研究，但对于最近描述的肌肉特异性激酶 (MuSK) 抗体介导的 MG 知之甚少。MuSK-MG 在临床上与 Ach-R MG 不同，对治疗的反应模式也不同，免疫发病机制也独特。
Zotero 插件