Department of Neurology, Graduate School of Medicine, Osaka University, Suita, Osaka, Japan; Department of Experimental Immunology, Immunology Frontier Research Center, Osaka University, Suita, Osaka, Japan; Integrated Frontier Research for Medical Science Division, Institute for Open and Transdisciplinary Research Initiatives (OTRI), Osaka University, Suita, Osaka, Japan; Department of Neurology, Yale School of Medicine, New Haven, CT, USA.
Department of Neurology, Graduate School of Medicine, Osaka University, Suita, Osaka, Japan.
Cell Rep. 2024 Sep 24;43(9):114677. doi: 10.1016/j.celrep.2024.114677. Epub 2024 Aug 23.
Myasthenia gravis (MG) is etiologically associated with thymus abnormalities, but its pathology in the thymus remains unclear. In this study, we attempt to narrow down the features associated with MG using spatial transcriptome analysis of thymoma and thymic hyperplasia samples. We find that the majority of thymomas are constituted by the cortical region. However, the small medullary region is enlarged in seropositive thymomas and contains polygenic enrichment and MG-specific germinal center structures. Neuromuscular medullary thymic epithelial cells, previously identified as MG-specific autoantigen-producing cells, are enriched in the cortico-medullary junction. The medulla is characterized by a specific chemokine pattern and immune cell composition, including migratory dendritic cells and effector regulatory T cells. Similar germinal center structures and immune microenvironments are also observed in the thymic hyperplasia medulla. This study shows that the medulla and junction areas are linked to MG pathology and provides insights into future MG research.
重症肌无力(MG)与胸腺异常有关,但胸腺的病理学仍不清楚。在这项研究中,我们试图使用胸腺瘤和胸腺增生样本的空间转录组分析来缩小与 MG 相关的特征。我们发现大多数胸腺瘤由皮质区域组成。然而,在血清阳性胸腺瘤中,小的髓质区域扩大,并包含多基因富集和 MG 特异性生发中心结构。神经肌肉髓质胸腺上皮细胞以前被鉴定为 MG 特异性自身抗原产生细胞,在皮质-髓质交界处富集。髓质的特点是具有特定的趋化因子模式和免疫细胞组成,包括迁移树突状细胞和效应调节性 T 细胞。胸腺增生的髓质中也观察到类似的生发中心结构和免疫微环境。这项研究表明,髓质和交界处与 MG 病理学有关,并为未来的 MG 研究提供了思路。