Central Laboratory, Affiliated Hospital of Jining Medical University, Jining, China.
Department of Hematology, Affiliated Hospital of Jining Medical University, Jining, China.
J Cell Mol Med. 2018 Sep;22(9):4496-4506. doi: 10.1111/jcmm.13761. Epub 2018 Jul 11.
Aplastic anaemia (AA) is a life-threatening hematopoietic disorder characterized by hypoplasia and pancytopenia with increasing fat cells in the bone marrow (BM). The BM-derived mesenchymal stem cells (MSCs) from AA are more susceptible to be induced into adipogenic differentiation compared with that from control, which may be causatively associated with the fatty BM and defective hematopoiesis of AA. Here in this study, we first demonstrated that levamisole displayed a significant suppressive effect on the in vitro adipogenic differentiation of AA BM-MSCs. Mechanistic investigation revealed that levamisole could increase the expression of ZFP36L1 which was subsequently demonstrated to function as a negative regulator of adipogenic differentiation of AA BM-MSCs through lentivirus-mediated ZFP36L1 knock-down and overexpression assay. Peroxisome proliferator-activated receptor gamma coactivator 1 beta (PPARGC1B) whose 3'-untranslated region bears adenine-uridine-rich elements was verified as a direct downstream target of ZFP36L1, and knock-down of PPARGC1B impaired the adipogenesis of AA BM-MSCs. Collectively, our work demonstrated that ZFP36L1-mediated post-transcriptional control of PPARGC1B expression underlies the suppressive effect of levamisole on the adipogenic differentiation of AA BM-MSCs, which not only provides novel therapeutic targets for alleviating the BM fatty phenomenon of AA patients, but also lays the theoretical and experimental foundation for the clinical application of levamisole in AA therapy.
再生障碍性贫血(AA)是一种危及生命的造血系统疾病,其特征是骨髓(BM)中细胞发育不良和全血细胞减少,脂肪细胞增多。与对照组相比,AA 患者来源的骨髓间充质干细胞(MSCs)更容易被诱导成脂肪细胞分化,这可能与 AA 患者的脂肪性 BM 和造血功能缺陷有关。在本研究中,我们首次证明了左旋咪唑对 AA 患者 BM-MSCs 的体外成脂分化具有显著的抑制作用。机制研究表明,左旋咪唑可以增加 ZFP36L1 的表达,随后通过慢病毒介导的 ZFP36L1 敲低和过表达实验证明,ZFP36L1 作为 AA BM-MSCs 成脂分化的负调控因子发挥作用。过氧化物酶体增殖物激活受体γ共激活因子 1β(PPARGC1B)的 3'非翻译区含有腺嘌呤-尿嘧啶丰富元件,被验证为 ZFP36L1 的直接下游靶标,敲低 PPARGC1B 会损害 AA BM-MSCs 的成脂分化。综上所述,我们的工作表明,ZFP36L1 介导的 PPARGC1B 表达的转录后调控是左旋咪唑抑制 AA BM-MSCs 成脂分化的基础,这不仅为缓解 AA 患者 BM 脂肪现象提供了新的治疗靶点,也为左旋咪唑在 AA 治疗中的临床应用奠定了理论和实验基础。