FLJ10849, a septin family gene, fuses MLL in a novel leukemia cell line CNLBC1 derived from chronic neutrophilic leukemia in transformation with t(4;11)(q21;q23)
FLJ10849, a septin family gene, fuses MLL in a novel leukemia cell line CNLBC1 derived from chronic neutrophilic leukemia in transformation with t(4;11)(q21;q23)
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DOI：
10.1038/sj.leu.2403334
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发表时间：
2004-05-01
期刊：
影响因子：
11.4
通讯作者：
Fujita, S
中科院分区：
文献类型：
--
作者：
Kojima, K;Sakai, I;Fujita, S
50-70% 的婴儿急性淋巴细胞白血病病例中描述了 t(4;11)(q21;q23)，而在儿童和成人急性淋巴细胞白血病和急性髓细胞白血病 (AML) 病例中则较少见。在 t(4;11)(q21;q23) 白血病中，AF4 基因已被克隆为 MLL 基因的融合伴侣。人髓系白血病细胞系慢性中性粒细胞白血病（CNL）BC1是从患有白血病转化的CNL患者的外周血标本中建立的。与原始白血病细胞一样，建立的细胞系具有 t(4;11)(q21;q23)。我们发现 11q23 上的 MLL 基因与 4q21 上的 FLJ10849 基因融合。 FLJ10849编码的蛋白属于septin家族，与人SEPT6具有最高同源性，SEPT6是t(X;11)(q13;q23) AML中MLL的融合伙伴之一。我们的结果表明，FLJ10849 可能定义了一个新的 septin 家族，特别参与 11q23 相关白血病的发病机制。所建立的细胞系CNLBC1可以为分析MLL-septin白血病和慢性中性粒细胞白血病的发病机制提供有用的模型。
A t(4;11)(q21;q23) has been described in 50-70% of cases of infant acute lymphoblastic leukemia and, less frequently, in cases of pediatric and adult acute lymphoblastic leukemia and acute myeloid leukemia (AML). In t(4;11)(q21;q23) leukemias, the AF4 gene has been cloned as a fusion partner of the MLL gene. A human myeloid leukemia cell line, chronic neutrophilic leukemia (CNL)BC1, was established from a peripheral blood specimen of a patient with CNL in leukemic transformation. As with the original leukemia cells, the established line had a t( 4; 11)( q21; q23). We showed that the MLL gene on 11q23 was fused to the FLJ10849 gene on 4q21. The protein encoded by FLJ10849 belongs to the septin family, sharing highest homology with human SEPT6, which is one of the fusion partners of MLL in t(X;11)(q13;q23) AML. Our results suggest that FLJ10849 might define a new septin family particularly involved in the pathogenesis of 11q23-associated leukemia. The established cell line, CNLBC1, could provide a useful model for analyzing the pathogenesis of MLL-septin leukemias and chronic neutrophilic leukemia.