临床儿科杂志 ›› 2018, Vol. 36 ›› Issue (11): 880-.doi: 10.3969/j.issn.1000-3606.2018.11.019

• 综合报道 • 上一篇    下一篇

儿童急性 B 淋巴细胞白血病中 IKAROS 家族锌指转录因子 1 基因异常的特征及临床意义#br#

闭军琴 1 综述, 包黎明 1,2 审校   

  1. 1.重庆医科大学附属儿童医院临床分子医学中心 儿童发育疾病研究教育部实验室 儿科学重庆市重点 实验室 重庆市儿童发育重大疾病诊治与预防国际科技合作基地(重庆 400014); 2.达特茅斯学院医学院病理检验系(美国新罕布什尔州 03756)
  • 收稿日期:2018-11-15 出版日期:2018-11-15 发布日期:2018-11-15

Features and clinical significance of IKZF1 aberrations in childhood B-cell acute lymphoblastic leukemia

Reviewer: BI Junqin1,  Reviser: BAO Liming1,2   

  1. 1.Center for Clinical Molecular Medicine,Ministry of Education Key Laboratory of Child Development Disorders, Chongqing Key Laboratory of Pediatrics, Children’ Hospital of Chongqing Medical University, Chongqing 400014, China; 2.Department of Pathology and Laboratory Medicine and Geisel School of Medicine at Dartmouth College, New Hampshire 03756, USA
  • Received:2018-11-15 Online:2018-11-15 Published:2018-11-15

摘要:  IKAROS家族锌指转录因子1(IKZF1)基因编码IKAROS蛋白,是一种重要的锌指样转录因子,在B淋巴细 胞增殖、分化及功能维持过程中发挥着重要的调控作用。IKZF1基因被认为是一个临床相关的肿瘤抑制因子,IKZF1缺失 致IKAROS单倍体剂量不足或显性负相与急性B淋巴细胞白血病(B-ALL)的发生发展紧密相关。因此,研究IKZF1基因 可为B-ALL的危险度分层提供新的分子标志物,有助于B-ALL个体化治疗。

Abstract:  IKAROS family zinc finger 1 (IKZF1) gene encoding a zinc finger transcription factor IKAROS plays an important role in the regulation of B lymphoid cell differentiation and proliferation. IKZF1 is a clinical tumor suppressive factor. Deletion of IKZF1 results in either haploinsufficiency or dominating negative for IKAROS that contributes to the development of B-cell acute lymphoblastic leukemia (B-ALL), associated with an poor prognosis. Study of IKZF1 may lead to identifying new risk stratification molecular indicator for B-ALL for a better guidance to individualized therapy.