Centre for Biological Systems Analysis, Albert-Ludwigs-University of Freiburg, Freiburg, Germany.
Leukemia. 2013 Jan;27(1):118-29. doi: 10.1038/leu.2012.222. Epub 2012 Aug 3.
Grb2-associated binder 2 (Gab2) serves as a critical amplifier in the signaling network of Bcr-Abl, the driver of chronic myeloid leukemia (CML). Despite the success of tyrosine kinase inhibitors (TKIs) in CML treatment, TKI resistance, caused by mutations in Bcr-Abl or aberrant activity of its network partners, remains a clinical problem. Using inducible expression and knockdown systems, we analyzed the role of Gab2 in Bcr-Abl signaling in human CML cells, especially with respect to TKI sensitivity. We show for the first time that Gab2 signaling protects CML cells from various Bcr-Abl inhibitors (imatinib, nilotinib, dasatinib and GNF-2), whereas Gab2 knockdown or haploinsufficiency leads to increased TKI sensitivity. We dissected the underlying molecular mechanism using various Gab2 mutants and kinase inhibitors and identified the Shp2/Ras/ERK and the PI3K/AKT/mTOR axes as the two critical signaling pathways. Gab2-mediated TKI resistance was associated with persistent phosphorylation of Gab2 Y452, a PI3K recruitment site, and consistent with this finding, the protective effect of Gab2 was completely abolished by the combination of dasatinib with the dual PI3K/mTOR inhibitor NVP-BEZ235. The identification of Gab2 as a novel modulator of TKI sensitivity in CML suggests that Gab2 could be exploited as a biomarker and therapeutic target in TKI-resistant disease.
Grb2 相关结合蛋白 2(Gab2)作为慢性髓系白血病(CML)驱动基因 Bcr-Abl 信号网络中的关键放大器。尽管酪氨酸激酶抑制剂(TKI)在 CML 治疗中取得了成功,但由于 Bcr-Abl 突变或其网络伙伴的异常活性导致的 TKI 耐药仍然是一个临床问题。我们使用诱导表达和敲低系统分析了 Gab2 在人类 CML 细胞中 Bcr-Abl 信号中的作用,特别是在 TKI 敏感性方面。我们首次表明,Gab2 信号可保护 CML 细胞免受各种 Bcr-Abl 抑制剂(伊马替尼、尼罗替尼、达沙替尼和 GNF-2)的影响,而 Gab2 敲低或杂合不足会导致 TKI 敏感性增加。我们使用各种 Gab2 突变体和激酶抑制剂剖析了潜在的分子机制,确定了 Shp2/Ras/ERK 和 PI3K/AKT/mTOR 轴作为两个关键信号通路。Gab2 介导的 TKI 耐药与 Gab2 Y452 的持续磷酸化有关,Gab2 Y452 是一个 PI3K 募集位点,与这一发现一致,用双重 PI3K/mTOR 抑制剂 NVP-BEZ235 与达沙替尼联合使用完全消除了 Gab2 的保护作用。Gab2 作为 CML 中 TKI 敏感性的新型调节剂的鉴定表明,Gab2 可以作为 TKI 耐药疾病的生物标志物和治疗靶点。