Department of Biochemistry, Chemistry and Pharmacy, Institute of Pharmaceutical Biology/ZAFES/Diagnostic Center of Acute Leukemia (DCAL), Goethe-University of Frankfurt, Frankfurt/Main, Germany.
Leukemia. 2013 Nov;27(11):2165-76. doi: 10.1038/leu.2013.135. Epub 2013 Apr 30.
Chromosomal rearrangements of the human MLL (mixed lineage leukemia) gene are associated with high-risk infant, pediatric, adult and therapy-induced acute leukemias. We used long-distance inverse-polymerase chain reaction to characterize the chromosomal rearrangement of individual acute leukemia patients. We present data of the molecular characterization of 1590 MLL-rearranged biopsy samples obtained from acute leukemia patients. The precise localization of genomic breakpoints within the MLL gene and the involved translocation partner genes (TPGs) were determined and novel TPGs identified. All patients were classified according to their gender (852 females and 745 males), age at diagnosis (558 infant, 416 pediatric and 616 adult leukemia patients) and other clinical criteria. Combined data of our study and recently published data revealed a total of 121 different MLL rearrangements, of which 79 TPGs are now characterized at the molecular level. However, only seven rearrangements seem to be predominantly associated with illegitimate recombinations of the MLL gene (≈ 90%): AFF1/AF4, MLLT3/AF9, MLLT1/ENL, MLLT10/AF10, ELL, partial tandem duplications (MLL PTDs) and MLLT4/AF6, respectively. The MLL breakpoint distributions for all clinical relevant subtypes (gender, disease type, age at diagnosis, reciprocal, complex and therapy-induced translocations) are presented. Finally, we present the extending network of reciprocal MLL fusions deriving from complex rearrangements.
人类 MLL(混合谱系白血病)基因的染色体重排与高危婴儿、儿科、成人和治疗诱导的急性白血病有关。我们使用长距离反转聚合酶链反应来描述单个急性白血病患者的染色体重排。我们提供了从急性白血病患者获得的 1590 个 MLL 重排活检样本的分子特征数据。确定了 MLL 基因内基因组断裂点的精确定位和涉及的易位伙伴基因(TPG),并确定了新的 TPG。所有患者均根据性别(852 名女性和 745 名男性)、诊断时的年龄(558 名婴儿、416 名儿科和 616 名成人白血病患者)和其他临床标准进行分类。我们的研究和最近发表的数据的综合数据显示,共有 121 种不同的 MLL 重排,其中 79 种 TPG 现在已在分子水平上得到了描述。然而,只有七种重排似乎与 MLL 基因的非法重组主要相关(约 90%):AFF1/AF4、MLLT3/AF9、MLLT1/ENL、MLLT10/AF10、ELL、部分串联重复(MLL PTDs)和 MLLT4/AF6。呈现了所有临床相关亚型(性别、疾病类型、诊断时的年龄、相互易位、复杂易位和治疗诱导易位)的 MLL 断点分布。最后,我们展示了源自复杂重排的相互 MLL 融合的扩展网络。