Molecular pathogenesis of core binding factor leukemia: current knowledge and future prospects

被引:61
作者
Goyama, Susumu [1 ]
Mulloy, James C. [1 ]
机构
[1] Univ Cincinnati, Coll Med, Cincinnati Childrens Hosp Med Ctr, Div Expt Hematol & Canc Biol, Cincinnati, OH 45221 USA
关键词
Core binding factor; AML1-ETO; CBF beta-MYH11; RUNX1; Acute myeloid leukemia; ACUTE MYELOID-LEUKEMIA; CBF-BETA-SMMHC; ACUTE MYELOGENOUS LEUKEMIA; T(8/21) FUSION PROTEIN; HUMAN CD34(+) CELLS; TUMOR-SUPPRESSOR; SELF-RENEWAL; C-KIT; TRANSCRIPTIONAL REPRESSION; EMBRYONIC LETHALITY;
D O I
10.1007/s12185-011-0858-z
中图分类号
R5 [内科学];
学科分类号
100201 [内科学];
摘要
Core binding factor (CBF) acute myeloid leukemia (AML) is the most common cytogenetic subtype of AML, defined by the presence of t(8;21) or inv(16)/t(16;16). The chromosomal aberrations create AML1-ETO and CBF beta-MYH11 fusion genes that disrupt the functions of CBF, an essential transcription factor in hematopoiesis. Despite the relatively good outcome of patients with CBF-AML, only approximately half of the patients are cured with current therapy, indicating the need for improved therapeutic strategies. In this review, we summarize current knowledge regarding altered transcriptional regulation, aberrant signaling pathways, and cooperating genetic events in CBF leukemia, and discuss challenges ahead for translating these findings into the clinic.
引用
收藏
页码:126 / 133
页数:8
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