Ranger A M, Oukka M, Rengarajan J, Glimcher L H
Department of Immunology and Infectious Diseases, Harvard School of Public Health, Boston, MA 02115-6017, USA.
Immunity. 1998 Nov;9(5):627-35. doi: 10.1016/s1074-7613(00)80660-3.
Nuclear factor of activated T cells (NFAT) is a critical regulator of early gene transcription in response to TCR-mediated signals. Here, we show that mice lacking both NFATp and NFAT4 develop a profound lymphoproliferative disorder likely due to a lowered threshold for TCR signaling coupled with increased resistance to apoptosis secondary to defective FasL expression. NFAT mutant mice also have allergic blepharitis, interstitial pneumonitis, and a 10(3) to 10(4) fold increase in serum IgG1 and IgE levels, secondary to a dramatic and selective increase in Th2 cytokines. This phenotype may be ascribed to unopposed occupancy of the IL-4 promoter by NFATc. Our data demonstrate that lymphoid homeostasis and Th2 activation require a critical balance among NFAT family members.
活化T细胞核因子(NFAT)是响应TCR介导信号的早期基因转录的关键调节因子。在此,我们表明,同时缺乏NFATp和NFAT4的小鼠会发生严重的淋巴细胞增殖性疾病,这可能是由于TCR信号阈值降低以及FasL表达缺陷导致的细胞凋亡抗性增加所致。NFAT突变小鼠还患有过敏性睑缘炎、间质性肺炎,并且由于Th2细胞因子急剧且选择性增加,血清IgG1和IgE水平增加了10³至10⁴倍。这种表型可能归因于NFATc对IL-4启动子的无对抗占据。我们的数据表明,淋巴细胞稳态和Th2激活需要NFAT家族成员之间的关键平衡。