Fusion of an AF4-related gene, LAF4, to MLL in childhood acute lymphoblastic leukemia with t(2;11)(q11;q23)
Fusion of an AF4-related gene, LAF4, to MLL in childhood acute lymphoblastic leukemia with t(2;11)(q11;q23)
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DOI：
10.1038/sj.onc.1206389
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发表时间：
2003-05-08
期刊：
影响因子：
8
通讯作者：
Hayashi, Y
中科院分区：
文献类型：
--
作者：
Hiwatari, M;Taki, T;Hayashi, Y
我们发现，在一名患有CD 10阳性急性淋巴细胞白血病（ALL）的儿童患者中，2q11.2-12上的LAF 4基因与11 q23上的MLL基因融合，该患者具有t（2;11）（q11;q23）。LAF 4基因编码具有反式激活潜力的1227个氨基酸的淋巴核蛋白，被认为在早期淋巴发育中起作用。LAF 4蛋白与婴儿早期pre-B ALL MLL融合蛋白AF 4和AF 5 q31同源，LAF 4的断裂点位于AF 4和AF 5 q31的反式激活结构域同源区域内。在成人心脏、脑、胎盘和胎儿脑中检测到8.5 kb LAF 4转录物的表达。LAF 4在ALL细胞系中的表达高于AML和EB病毒转化的B淋巴细胞系。这些发现表明，LAF 4，AF 4和AF 5 q31可能定义了一个新的家庭，特别是参与11 q23相关的ALL的发病机制。
We showed that the LAF4 gene on 2q11.2-12 was fused to the MLL gene on 11q23 in a pediatric patient with CD10 positive acute lymphoblastic leukemia (ALL) having t(2;11)(q11;q23). The LAF4 gene, which encodes a lymphoid nuclear protein of 1227 amino acids with transactivation potential, is thought to have a role in early lymphoid development. The LAF4 protein was homologous to AF4 and AF5q31 proteins that are fused to MLL in infant early pre-B ALL and the breakpoint of LAF4 was located within the region homologous to the transactivation domain of AF4 and AF5q31. Expression of the 8.5-kb LAF4 transcript was detected in the adult heart, brain, and placenta and in the fetal brain. LAF4 expression was found to be higher in ALL cell lines than in AML and Epstein-Barr virus-transformed B-lymphocyte cell lines. These findings suggest that LAF4, AF4 and AF5q31 might define a new family particularly involved in the pathogenesis of 11q23-associated ALL.