Schoumans Jacqueline, Johansson Bertil, Corcoran Martin, Kuchinskaya Ekaterina, Golovleva Irina, Grandér Dan, Forestier Erik, Staaf Johan, Borg Ake, Gustafsson Britt, Blennow Elisabeth, Nordgren Ann
Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden.
Br J Haematol. 2006 Nov;135(4):492-9. doi: 10.1111/j.1365-2141.2006.06328.x. Epub 2006 Sep 26.
Although the dic(9;20)(p11-13;q11) is a recurrent chromosomal abnormality in paediatric B-cell precursor acute lymphoblastic leukaemia (BCP ALL), occurring in approximately 2% of the cases, its molecular genetic consequences have not been elucidated. In the present study, high-resolution genome-wide array-based comparative genomic hybridisation (array-CGH) and fluorescence in situ hybridisation (FISH) were used to characterise the 9p and 20q breakpoints (BPs) in seven childhood BCP ALLs with dic(9;20), which was shown to be unbalanced in all of them, resulting in loss of 9p13.2-pter. Five of the cases had loss of 20q11.2-qter, whereas two displayed gain of 20cen-pter. All BPs on 9p clustered in a 1.5 Mb segment of the sub-band 9p13.2; in three of the cases, the 20q BPs mapped to three adjacent clones covering a distance of 350 kb at 20q11.2. Thus, the aberration should be designated dic(9;20)(p13.2;q11.2). One of the ALLs, shown to have a complex dic(9;20), was further investigated by FISH, revealing a rearrangement of the haemapoietic cell kinase isoform p61 (HCK) gene at 20q11. The disruption of HCK may result in a fusion gene or in loss of function. Unfortunately, lack of material precluded further analyses of HCK. Thus, it remains to be elucidated whether dic(9;20)(p13.2;q11.2) leads to a chimaeric gene or whether the functionally important outcome is loss of 9p and 20q material.
尽管dic(9;20)(p11 - 13;q11)是小儿B细胞前体急性淋巴细胞白血病(BCP ALL)中一种常见的染色体异常,约2%的病例会出现,但尚未阐明其分子遗传学后果。在本研究中,使用高分辨率全基因组基于阵列的比较基因组杂交(阵列CGH)和荧光原位杂交(FISH)对7例患有dic(9;20)的儿童BCP ALL中的9p和20q断点(BP)进行了特征分析,结果显示所有病例均为不平衡型,导致9p13.2 - pter缺失。其中5例病例出现20q11.2 - qter缺失,而2例显示20cen - pter增益。9p上的所有BP都聚集在9p13.2亚带的1.5 Mb片段中;在3例病例中,20q BP定位于20q11.2处覆盖350 kb距离的三个相邻克隆。因此,该畸变应命名为dic(9;20)(p13.2;q11.2)。其中1例ALL显示具有复杂的dic(9;20),通过FISH进一步研究发现20q11处的造血细胞激酶异构体p61(HCK)基因发生重排。HCK的破坏可能导致融合基因或功能丧失。不幸的是,由于材料不足无法对HCK进行进一步分析。因此,dic(9;20)(p13.2;q11.2)是否导致嵌合基因,或者功能上重要的结果是否是9p和20q物质的缺失,仍有待阐明。