Inflammatory processes in cortical tubers and subependymal giant cell tumors of tuberous sclerosis complex
Inflammatory processes in cortical tubers and subependymal giant cell tumors of tuberous sclerosis complex
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DOI：
10.1016/j.eplepsyres.2007.10.002
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发表时间：
2008-01-01
影响因子：
2.2
通讯作者：
Aronica, E.
中科院分区：
文献类型：
--
作者：
Boer, K.;Jansen, F.;Aronica, E.
皮质结节和室管膜下巨细胞瘤（SGCT）是两种主要的脑肿瘤。结节性硬化症（TSC）相关病变。在本研究中，我们研究了免疫细胞化学的炎症细胞成分和诱导的两个主要的促炎途径（白细胞介素（IL）-1 β和补体途径）的块茎和SGCT切除TSC患者。所有病变的特征在于表达II类抗原（HLA-DR）的小胶质细胞的突出存在，并在较小程度上，CD 68阳性巨噬细胞的存在。我们还观察到血管周围和实质T淋巴细胞（CD 3（+）），以CD 8（+）T细胞毒性/抑制性淋巴细胞为主。活化的小胶质细胞和反应性星形胶质细胞表达IL-1 β及其信号受体IL-1 RI，以及补体级联反应的组分，如C1 q、C3 c和C3 d。在块茎和SGCT中均观察到白蛋白外渗和星形胶质细胞摄取，表明血脑屏障通透性的改变与TSC相关病变中的炎症相关。我们的研究结果表明，在皮质结节和SGCT的炎症通路的持续和复杂的激活。(C)2007 Elsevier B. V.保留所有权利。
Cortical tubers and subependymal giant cell tumors (SGCT) are two major cerebral. lesions associated with tuberous sclerosis complex (TSC). In the present study, we investigated immunocytochemically the inflammatory cell components and the induction of two major pro-inflammatory pathways (the interleukin (IL)-1 beta and complement pathways) in tubers and SGCT resected from TSC patients. All lesions were characterized by the prominent presence of microglial cells expressing class II-antigens (HLA-DR) and, to a lesser extent, the presence of CD68-positive macrophages. We also observed perivascular and parenchymal T lymphocytes (CD3(+)) with a predominance of CD8(+) T-cytotoxic/suppressor lymphoid cells. Activated microglia and reactive astrocytes expressed IL-1 beta and its signaling receptor IL-1RI, as well as components of the complement cascade, such as C1q, C3c and C3d. Albumin extravasation, with uptake in astrocytes, was observed in both tubers and SGCT, suggesting that alterations in blood brain barrier permeability are associated with inflammation in TSC-associated lesions. Our findings demonstrate a persistent and complex activation of inflammatory pathways in cortical tubers and SGCT. (C) 2007 Elsevier B.V. All rights reserved.