Heikenwalder Mathias, Prinz Marco, Zeller Nicolas, Lang Karl S, Junt Tobias, Rossi Simona, Tumanov Alexei, Schmidt Hauke, Priller Josef, Flatz Lukas, Rülicke Thomas, Macpherson Andrew J, Holländer Georg A, Nedospasov Sergei A, Aguzzi Adriano
Institute of Neuropathology, University Hospital of Zürich, Schmelzbergstrasse 12, CH-8091 Zürich, Switzerland.
Am J Pathol. 2008 Jun;172(6):1555-70. doi: 10.2353/ajpath.2008.070572. Epub 2008 May 15.
Activated lymphocytes and lymphoid-tissue inducer cells express lymphotoxins (LTs), which are essential for the organogenesis and maintenance of lymphoreticular microenvironments. Here we describe that T-cell-restricted overexpression of LT induces fulminant thymic involution. This phenotype was prevented by ablation of the LT receptors tumor necrosis factor receptor (TNFR) 1 or LT beta receptor (LTbetaR), representing two non-redundant pathways. Multiple lines of transgenic Ltalphabeta and Ltalpha mice show such a phenotype, which was not observed on overexpression of LTbeta alone. Reciprocal bone marrow transfers between LT-overexpressing and receptor-ablated mice show that involution was not due to a T cell-autonomous defect but was triggered by TNFR1 and LTbetaR signaling to radioresistant stromal cells. Thymic involution was partially prevented by the removal of one allele of LTbetaR but not of TNFR1, establishing a hierarchy in these signaling events. Infection with the lymphocytic choriomeningitis virus triggered a similar thymic pathology in wt, but not in Tnfr1(-/-) mice. These mice displayed elevated TNFalpha in both thymus and plasma, as well as increased LTs on both CD8(+) and CD4(-)CD8(-) thymocytes. These findings suggest that enhanced T cell-derived LT expression helps to control the physiological size of the thymic stroma and accelerates its involution via TNFR1/LTbetaR signaling in pathological conditions and possibly also in normal aging.
活化的淋巴细胞和淋巴组织诱导细胞表达淋巴毒素(LTs),这对于淋巴网状微环境的器官发生和维持至关重要。在此我们描述,T细胞限制性过表达LT会诱导暴发性胸腺退化。这种表型可通过去除LT受体肿瘤坏死因子受体(TNFR)1或LTβ受体(LTβR)来预防,这代表了两条非冗余途径。多系转基因Ltαβ和Ltα小鼠表现出这种表型,而单独过表达LTβ时未观察到这种表型。在LT过表达小鼠和受体缺失小鼠之间进行相互骨髓移植表明,退化并非由于T细胞自主性缺陷,而是由TNFR1和LTβR向放射抗性基质细胞的信号传导触发的。去除一个LTβR等位基因可部分预防胸腺退化,但去除TNFR1等位基因则不能,这在这些信号事件中建立了一个等级体系。感染淋巴细胞性脉络丛脑膜炎病毒在野生型小鼠中引发了类似的胸腺病理变化,但在Tnfr1(-/-)小鼠中未引发。这些小鼠的胸腺和血浆中TNFα水平升高,CD8(+)和CD4(-)CD8(-)胸腺细胞上的LTs也增加。这些发现表明,增强的T细胞源性LT表达有助于控制胸腺基质的生理大小,并在病理条件下以及可能在正常衰老过程中通过TNFR1/LTβR信号传导加速其退化。