Zhao Ping, Hall Jacob, Durston Mary, Voydanoff Austin, VanSickle Elizabeth, Kelly Shannon, Nagulapally Abhinav B, Bond Jeffery, Saulnier Sholler Giselle
Pediatric Oncology Translational Research Program, Helen DeVos Children's Hospital, Grand Rapids, MI, United States of America.
Calvin College, Grand Rapids, MI, United States of America.
PLoS One. 2017 Jun 29;12(6):e0179948. doi: 10.1371/journal.pone.0179948. eCollection 2017.
Medulloblastoma (MB) is the most common malignant brain tumor in children, accounting for nearly 20 percent of all childhood brain tumors. New treatment strategies are needed to improve patient survival outcomes and to reduce adverse effects of current therapy. The phosphatidylinositol-3-kinase (PI3K)/AKT/mammalian target of rapamycin (mTOR) intracellular signaling pathway plays a key role in cellular metabolism, proliferation, survival and angiogenesis, and is often constitutively activated in human cancers, providing unique opportunities for anticancer therapeutic intervention. The aim of this study was to evaluate the pre-clinical activity of BKM120, a selective pan-class I PI3K inhibitor, on MB cell lines and primary samples. IC50 values of BKM120 in the twelve MB cell lines tested ranged from 0.279 to 4.38 μM as determined by cell viability assay. IncuCyte ZOOM Live-Cell Imaging system was used for kinetic monitoring of cytotoxicity of BKM120 and apoptosis in MB cells. BKM120 exhibited cytotoxicity in MB cells in a dose and time-dependent manner by inhibiting activation of downstream signaling molecules AKT and mTOR, and activating caspase-mediated apoptotic pathways. Furthermore, BKM120 decreased cellular glycolytic metabolic activity in MB cell lines in a dose-dependent manner demonstrated by ATP level per cell. In MB xenograft mouse study, DAOY cells were implanted in the flank of nude mice and treated with vehicle, BKM120 at 30 mg/kg and 60 mg/kg via oral gavage daily. BKM120 significantly suppressed tumor growth and prolonged mouse survival. These findings help to establish a basis for clinical trials of BKM120, which could be a novel therapy for the treatment of medulloblastoma patients.
髓母细胞瘤(MB)是儿童中最常见的恶性脑肿瘤,占所有儿童脑肿瘤的近20%。需要新的治疗策略来改善患者的生存结局并减少当前治疗的不良反应。磷脂酰肌醇-3-激酶(PI3K)/蛋白激酶B(AKT)/雷帕霉素哺乳动物靶蛋白(mTOR)细胞内信号通路在细胞代谢、增殖、存活和血管生成中起关键作用,并且在人类癌症中常常组成性激活,为抗癌治疗干预提供了独特的机会。本研究的目的是评估选择性泛I类PI3K抑制剂BKM120对MB细胞系和原代样本的临床前活性。通过细胞活力测定法确定,BKM120在测试的12种MB细胞系中的半数抑制浓度(IC50)值范围为0.279至4.38μM。使用IncuCyte ZOOM活细胞成像系统对BKM120的细胞毒性和MB细胞中的凋亡进行动力学监测。BKM120通过抑制下游信号分子AKT和mTOR的激活以及激活半胱天冬酶介导的凋亡途径,以剂量和时间依赖性方式在MB细胞中表现出细胞毒性。此外,BKM120以剂量依赖性方式降低了MB细胞系中的细胞糖酵解代谢活性,这通过每个细胞的三磷酸腺苷(ATP)水平得以证明。在MB异种移植小鼠研究中,将DAOY细胞植入裸鼠的胁腹,并通过每日口服灌胃给予溶剂、30mg/kg和60mg/kg的BKM120。BKM120显著抑制肿瘤生长并延长小鼠存活时间。这些发现有助于为BKM120的临床试验建立基础,BKM120可能是治疗髓母细胞瘤患者的一种新疗法。