Division of Hematology and Oncology, Department of Medicine, Kyoto Prefectural University of Medicine, 465 Kajii-cho, Kamigyo-ku, Kyoto, 602-8566, Japan.
Apoptosis. 2013 Nov;18(11):1437-1446. doi: 10.1007/s10495-013-0882-y.
PP2A activator FTY720 has been shown to possess the anti-leukemic activity for chronic myelogenous leukemia (CML), however, the cell killing mechanism underlying its anti-leukemic activity has remained to be verified. We investigated the precise mechanisms underlying the apoptosis induction by FTY720, especially focusing on the roles of BH3-only proteins, and the therapeutic potency of FTY720 for CML. Enforced expression of either BCL2 or the dominant-negative protein of FADD (FADD.DN) partly protected CML cells from apoptosis by FTY720, indicating the involvement of both cell extrinsic and intrinsic apoptosis pathways. FTY720 activates pro-apoptotic BH3-only proteins: BIM, which is essential for apoptosis by BCR-ABL1 tyrosine kinase inhibitors (TKIs), and BID, which accelerates the extrinsic apoptosis pathway. Gene knockdown of either BIM or BID partly protected K562 cells from apoptosis by FTY720, but the extent of cell protection was not as much as that by overexpression of either BCL2 or FADD.DN. Moreover, knockdown of both BIM and BID did not provide additional protection compared with knockdown of only BIM or BID, indicating that BIM and BID complement each other in apoptosis by FTY720, especially when either is functionally impaired. FTY720 can overcome TKI resistance caused by ABL kinase domain mutations, dysfunction of BIM resulting from gene deletion polymorphism, and galectin-3 overexpression. In addition, ABT-263, a BH3-mimetic, significantly augmented cell death induction by FTY720 both in TKI-sensitive and -resistant leukemic cells. These results provide the rationale that FTY720, with its unique effects on BIM and BID, could lead to new therapeutic strategies for CML.
蛋白磷酸酶 2A 激活剂 FTY720 已被证明具有抗慢性髓系白血病(CML)的活性,但其抗白血病活性的细胞杀伤机制仍有待验证。我们研究了 FTY720 诱导细胞凋亡的确切机制,特别是重点研究了 BH3 仅蛋白的作用,以及 FTY720 治疗 CML 的疗效。过表达 BCL2 或 FADD 的显性失活蛋白(FADD.DN)部分保护 CML 细胞免受 FTY720 的凋亡,表明同时涉及细胞外和细胞内凋亡途径。FTY720 激活促凋亡 BH3 仅蛋白:BCR-ABL1 酪氨酸激酶抑制剂(TKI)诱导凋亡所必需的 BIM,以及加速细胞外凋亡途径的 BID。敲低 BIM 或 BID 均可部分保护 K562 细胞免受 FTY720 的凋亡,但细胞保护程度不如过表达 BCL2 或 FADD.DN。此外,与仅敲低 BIM 或 BID 相比,同时敲低 BIM 和 BID 并未提供额外的保护,表明 BIM 和 BID 在 FTY720 诱导的凋亡中相互补充,尤其是当其中一种功能受损时。FTY720 可以克服 ABL 激酶结构域突变引起的 TKI 耐药、基因缺失多态性导致的 BIM 功能障碍和半乳糖凝集素-3 过表达引起的 TKI 耐药。此外,BH3 模拟物 ABT-263 显著增强了 TKI 敏感和耐药白血病细胞中 FTY720 诱导的细胞死亡。这些结果为 FTY720 通过其对 BIM 和 BID 的独特作用,为 CML 提供了新的治疗策略提供了依据。