Cancer Center, Beijing Shijitan Hospital, Capital Medical Hospital, Beijing, China.
Clinical Department, Capital Medical University, Beijing, China.
Cancer Gene Ther. 2015 Dec;22(12):573-80. doi: 10.1038/cgt.2015.50. Epub 2015 Oct 23.
The p53 mutation in chronic myeloid leukemia (CML) led to decreased overall survival and therapy resistance which was also closely correlated with the downstream proto-oncogenes BCL-2, TCL-1 and MCL-1. We in this study aimed to investigate the function of miR130a in p53 tumor suppressor signaling pathway. We performed microRNA (miRNA) expression profile analysis in CML cancer stem cells of 38 cases and extracted total RNA from peripheral blood of 143 cases. Standard curves of U6 and miRNA were made from 10-fold serial dilutions of the cDNA, which were quantified using real-time quantitative PCR with SYBR Green by ABI 7300. The p53 mutations and BCR/ABL mutation status analysis in CML patients were detected by PCR and direct sequencing. Candidate targets of miR130a of putative relevance in CML pathogenesis were analyzed by bioinformatics approach. We then used dual-luciferase activity assay to verify the target genes of miR130a and used western blot analysis to elucidate the mechanism of miR130a on modulating drug resistance. The levels of miR-130a expression in CML were significantly lower in poor prognostic subgroups, defined by prognostic factors including mutated BCR/ABL status, p53 and ATM deletions and p53 mutations. Furthermore, underexpression of miR-130a was significantly associated with shorter overall survival and treatment-free survival in CML patients. We demonstrated that miR130a function as tumor suppressors by inhibiting multiple anti-apoptosis proteins, including BCL-2, MCL-1 and XIAP. This was a direct effect because miR130a negatively regulated expression of a BCL-2/MCL-1/XIAP 3'untranslated region-based reporter construct. Transfection of miR130a mimics into CML cells from 30 patients without p53 aberrations led to significant increases in apoptosis compared with transfection with the miRNA control. Besides, enforced expression of miR130a had no significant drug-sensitization effect in CML cells from p53-attenuated patients. MiR-130a may have an important role in the pathogenesis of CML and may be useful for assessing prognosis in patients with CML. Moreover, miR130a may provide a possible therapeutic avenue and a sensitive indicator of the activity of the p53 in CML.
慢性髓性白血病(CML)中的 p53 突变导致总生存期缩短和治疗耐药,这与下游原癌基因 BCL-2、TCL-1 和 MCL-1 密切相关。本研究旨在探讨 miR130a 在 p53 肿瘤抑制信号通路中的功能。我们对 38 例 CML 癌干细胞进行了 microRNA(miRNA)表达谱分析,并从 143 例患者的外周血中提取总 RNA。通过对 cDNA 进行 10 倍系列稀释,制作 U6 和 miRNA 的标准曲线,然后使用 ABI 7300 上的实时定量 PCR 和 SYBR Green 对其进行定量。通过 PCR 和直接测序检测 CML 患者的 p53 突变和 BCR/ABL 突变状态。通过生物信息学方法分析与 CML 发病机制相关的 miR130a 的潜在靶标。然后使用双荧光素酶活性测定来验证 miR130a 的靶基因,并使用 Western blot 分析来阐明 miR130a 调节耐药性的机制。在预后不良的亚组中,miR-130a 的表达水平显著降低,这些亚组由包括突变的 BCR/ABL 状态、p53 和 ATM 缺失以及 p53 突变在内的预后因素定义。此外,miR-130a 的低表达与 CML 患者的总生存期和无治疗生存期较短显著相关。我们证明 miR130a 通过抑制多种抗凋亡蛋白,包括 BCL-2、MCL-1 和 XIAP,发挥肿瘤抑制因子的作用。这是一种直接作用,因为 miR130a 负调控基于 BCL-2/MCL-1/XIAP 3'UTR 的报告构建体的表达。与转染 miRNA 对照相比,将 miR130a 模拟物转染到来自 30 名无 p53 异常的 CML 患者的细胞中,导致细胞凋亡显著增加。此外,在 p53 减弱的患者的 CML 细胞中,强制表达 miR130a 对药物无明显增敏作用。miR-130a 可能在 CML 的发病机制中发挥重要作用,并可能有助于评估 CML 患者的预后。此外,miR130a 可能为 CML 提供一种可能的治疗途径和 p53 活性的敏感指标。