重症肌无力的胸腺生理与病理生理。

International review of neurobiology Pub Date : 2025-01-01 Epub Date: 2025-05-12 DOI:10.1016/bs.irn.2025.04.023
Nadine Dragin, Rozen Le Panse
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引用次数: 0

摘要

胸腺是负责t细胞发育和成熟的中枢淋巴器官,在适应性免疫中至关重要。这个器官为胸腺细胞分化和阳性/阴性选择创造了一个特殊的环境,确保功能性和自我耐受的T细胞的生存,同时消除自身反应性克隆。这一过程被称为T细胞教育,涉及发育中的T细胞和基质细胞(主要是胸腺上皮细胞)之间的相互作用,基质细胞提供组织特异性抗原。适当的胸腺功能对维持免疫稳态至关重要。然而,胸腺异常与重症肌无力(MG)有关,胸腺常表现为淋巴滤泡增生或胸腺瘤,这可能引发神经肌肉连接处针对乙酰胆碱受体的自身免疫反应。这导致神经肌肉传递受损和特征性肌肉无力。了解与MG相关的胸腺改变的病因机制对于阐明由胸腺异常引起的免疫失调至关重要,这种异常甚至可能在治疗性胸腺切除术后持续存在。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Thymic physiology and pathophysiology in Myasthenia Gravis.

The thymus is a central lymphoid organ responsible for T-cell development and maturation and is crucial in adaptive immunity. This organ creates a specialized environment for thymocyte differentiation and positive/negative selection, ensuring the survival of functional and self-tolerant T cells while eliminating autoreactive clones. This process, which is known as T-cell education, involves interactions between developing T cells and stromal cells, primarily thymic epithelial cells, which present tissue-specific antigens. Proper thymus function remains vital for maintaining immune homeostasis. However, thymic abnormalities have been implicated in Myasthenia Gravis (MG), where the thymus often exhibits lymphofollicular hyperplasia or thymomas, which may trigger an autoimmune response against the acetylcholine receptor at the neuromuscular junction. This leads to impaired neuromuscular transmission and characteristic muscle weakness. Understanding the etiological mechanisms underlying thymic alterations associated with MG is crucial for elucidating immune dysregulation resulting from an abnormal thymus, which may persist even post-therapeutic thymectomy.

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