Staber Philipp B, Vesely Paul, Haq Naznin, Ott Rene G, Funato Kotaro, Bambach Isabella, Fuchs Claudia, Schauer Silvia, Linkesch Werner, Hrzenjak Andelko, Dirks Wilhelm G, Sexl Veronika, Bergler Helmut, Kadin Marshall E, Sternberg David W, Kenner Lukas, Hoefler Gerald
Klinische Abteilung für Hämatologie, Universitätsklinik für Innere Medizin, Medizinische, Universität Graz, Auenbruggerplatz 38, A-8036 Graz, Austria.
Blood. 2007 Nov 1;110(9):3374-83. doi: 10.1182/blood-2007-02-071258. Epub 2007 Aug 9.
Anaplastic large cell lymphomas (ALCLs) are highly proliferating tumors that commonly express the AP-1 transcription factor JunB. ALK fusions occur in approximately 50% of ALCLs, and among these, 80% have the t(2;5) translocation with NPM-ALK expression. We report greater activity of JunB in NPM-ALK-positive than in NPM-ALK-negative ALCLs. Specific knockdown of JUNB mRNA using small interfering RNA and small hairpin RNA in NPM-ALK-expressing cells decreases cellular proliferation as evidenced by a reduced cell count in the G2/M phase of the cell cycle. Expression of NPM-ALK results in ERK1/2 activation and transcriptional up-regulation of JUNB. Both NPM-ALK-positive and -negative ALCL tumors demonstrate active ERK1/2 signaling. In contrast to NPM-ALK-negative ALCL, the mTOR pathway is active in NPM-ALK-positive lymphomas. Pharmacological inhibition of mTOR in NPM-ALK-positive cells down-regulates JunB protein levels by shifting JUNB mRNA translation from large polysomes to monosomes and ribonucleic particles (RNPs), and decreases cellular proliferation. Thus, JunB is a critical target of mTOR and is translationally regulated in NPM-ALK-positive lymphomas. This is the first study demonstrating translational control of AP-1 transcription factors in human neoplasia. In conjunction with NPM-ALK, JunB enhances cell cycle progression and may therefore represent a therapeutic target.
间变性大细胞淋巴瘤(ALCL)是高度增殖性肿瘤,通常表达AP-1转录因子JunB。ALK融合在约50%的ALCL中出现,其中80%具有t(2;5)易位并表达NPM-ALK。我们报告JunB在NPM-ALK阳性的ALCL中比在NPM-ALK阴性的ALCL中具有更高的活性。在表达NPM-ALK的细胞中使用小干扰RNA和小发夹RNA特异性敲低JUNB mRNA可降低细胞增殖,这在细胞周期G2/M期细胞计数减少中得到证实。NPM-ALK的表达导致ERK1/2激活和JUNB的转录上调。NPM-ALK阳性和阴性的ALCL肿瘤均显示ERK1/2信号活跃。与NPM-ALK阴性的ALCL相反,mTOR途径在NPM-ALK阳性淋巴瘤中活跃。对NPM-ALK阳性细胞中mTOR的药理抑制通过将JUNB mRNA翻译从大多核糖体转移到单核糖体和核糖核蛋白颗粒(RNP)来下调JunB蛋白水平,并降低细胞增殖。因此,JunB是mTOR的关键靶点,并且在NPM-ALK阳性淋巴瘤中受到翻译调控。这是第一项证明人类肿瘤中AP-1转录因子存在翻译控制的研究。与NPM-ALK一起,JunB增强细胞周期进程,因此可能代表一个治疗靶点。