Rahmani Mohamed, Aust Mandy Mayo, Hawkins Elisa, Parker Rebecca E, Ross Masey, Kmieciak Maciej, Reshko Leonid Borisovich, Rizzo Kathryn A, Dumur Catherine I, Ferreira-Gonzalez Andrea, Grant Steven
Department of Medicine, Virginia Commonwealth University, the Virginia Institute for Molecular Medicine, and the Massey Cancer Center, Richmond, VA, USA.
Department of Pathology, Virginia Commonwealth University, the Virginia Institute for Molecular Medicine, and the Massey Cancer Center, Richmond, VA, USA.
Haematologica. 2015 Dec;100(12):1553-63. doi: 10.3324/haematol.2015.130351. Epub 2015 Oct 9.
Effects of concurrent inhibition of mTORC1/2 and Bcl-2/Bcl-xL in human acute myeloid leukemia cells were examined. Tetracycline-inducible Bcl-2/Bcl-xL dual knockdown markedly sensitized acute myeloid leukemia cells to the dual TORC1/2 inhibitor INK128 in vitro as well as in vivo. Moreover, INK128 co-administered with the Bcl-2/xL antagonist ABT-737 sharply induced cell death in multiple acute myeloid leukemia cell lines, including TKI-resistant FLT3-ITD mutants and primary acute myeloid leukemia blasts carrying various genetic aberrations e.g., FLT3, IDH2, NPM1, and Kras, while exerting minimal toxicity toward normal hematopoietic CD34(+) cells. Combined treatment was particularly active against CD34(+)/CD38(-)/CD123(+) primitive leukemic progenitor cells. The INK128/ABT-737 regimen was also effective in the presence of a protective stromal microenvironment. Notably, INK128 was more potent than the TORC1 inhibitor rapamycin in down-regulating Mcl-1, diminishing AKT and 4EBP1 phosphorylation, and potentiating ABT-737 activity. Mcl-1 ectopic expression dramatically attenuated INK128/ABT-737 lethality, indicating an important functional role for Mcl-1 down-regulation in INK128/ABT-737 actions. Immunoprecipitation analysis revealed that combined treatment markedly diminished Bax, Bak, and Bim binding to all major anti-apoptotic Bcl-2 members (Bcl-2/Bcl-xL/Mcl-1), while Bax/Bak knockdown reduced cell death. Finally, INK128/ABT-737 co-administration sharply attenuated leukemia growth and significantly prolonged survival in a systemic acute myeloid leukemia xenograft model. Analysis of subcutaneous acute myeloid leukemia-derived tumors revealed significant decrease in 4EBP1 phosphorylation and Mcl-1 protein level, consistent with results obtained in vitro. These findings demonstrate that co-administration of dual mTORC1/mTORC2 inhibitors and BH3-mimetics exhibits potent anti-leukemic activity in vitro and in vivo, arguing that this strategy warrants attention in acute myeloid leukemia.
研究了同时抑制mTORC1/2和Bcl-2/Bcl-xL对人急性髓系白血病细胞的影响。四环素诱导的Bcl-2/Bcl-xL双敲低在体外和体内均显著增强了急性髓系白血病细胞对双TORC1/2抑制剂INK128的敏感性。此外,INK128与Bcl-2/xL拮抗剂ABT-737联合给药可在多种急性髓系白血病细胞系中急剧诱导细胞死亡,包括对酪氨酸激酶抑制剂(TKI)耐药的FLT3-ITD突变体以及携带各种基因异常(如FLT3、IDH2、NPM1和Kras)的原发性急性髓系白血病母细胞,同时对正常造血CD34(+)细胞的毒性最小。联合治疗对CD34(+)/CD38(-)/CD123(+)原始白血病祖细胞特别有效。在存在保护性基质微环境的情况下,INK128/ABT-737方案也有效。值得注意的是,INK128在下调Mcl-1、减少AKT和4EBP1磷酸化以及增强ABT-737活性方面比TORC1抑制剂雷帕霉素更有效。Mcl-1异位表达显著减弱了INK128/ABT-737的致死性,表明Mcl-1下调在INK128/ABT-737作用中具有重要的功能作用。免疫沉淀分析显示,联合治疗显著减少了Bax、Bak和Bim与所有主要抗凋亡Bcl-2成员(Bcl-2/Bcl-xL/Mcl-1)的结合,而Bax/Bak敲低减少了细胞死亡。最后,在系统性急性髓系白血病异种移植模型中,INK128/ABT-737联合给药显著减弱了白血病生长并显著延长了生存期。对皮下急性髓系白血病衍生肿瘤的分析显示,4EBP1磷酸化和Mcl-1蛋白水平显著降低,与体外实验结果一致。这些发现表明,双mTORC1/mTORC2抑制剂和BH-3模拟物联合给药在体外和体内均表现出强大的抗白血病活性,表明该策略在急性髓系白血病中值得关注。