摘要
CCAAT增强子结合蛋白A(CEBPA)基因及其编码的转录因子CCAAT增强子结合蛋白α(C/EBPα)通过促进造血干/祖细胞向粒系分化并抑制细胞增殖,在造血系统早期分化过程中起重要作用。CEBPA基因的突变及其在转录、翻译及翻译后水平受到的异常调控可引起C/EBPα蛋白结构或表达异常,导致粒系分化成熟障碍、不成熟粒系前体细胞异常增殖,是急性髓性白血病(AML)的重要发病机制。CEBPA基因突变和表达异常调节对AML患者的预后有重要影响,并可作为诱导分化治疗的靶点。本文就CEBPA基因突变与AML、C/EBPα蛋白表达调控异常与AML及C/EBPα蛋白与靶向治疗进行综述。
CCAAT enhancer binding protein A（CEBPA） and its product transcription factor CCAAT enhancer binding protein α（C/EBPα） play pivotal roles in early granulocyte development.C/EBPα induces the transition and keeps the balance of differentiation and proliferation of myeloid progenitors.The mutation and dysregulation of CEBPA at transcription,translation or post-translation level lead to differentiation block and over proliferation of immature hematopoietic cells,which are important mechanisms of acute myeloid leukemia（AML）.The mutation and dysregulation of CEBPA also provide clues for evaluating the outcome of AML patients and potential targets for differentiation-inducing therapies.This review focus on CEBPA mutation and AML,dysregulation of C/EBPα protein expression and AML,as well as C/EBPα protein and targeting therapy.
出处
《中国实验血液学杂志》
CAS
CSCD
北大核心
2012年第5期1256-1260,共5页
Journal of Experimental Hematology
基金
国家自然科学基金资助项目(编号81170501)
