Haluska F G, Tsujimoto Y, Croce C M
Wistar Institute, Philadelphia, PA 19104.
Proc Natl Acad Sci U S A. 1987 Oct;84(19):6835-9. doi: 10.1073/pnas.84.19.6835.
Recent molecular analyses of Burkitt lymphomas carrying the t(8;14) chromosome translocation have indicated that a dichotomy exists regarding the molecular mechanisms by which the translocations occur. Most sporadic Burkitt tumors carry translocations that apparently arise due to mistakes in the immunoglobulin isotype-switching process. In contrast, there is evidence that the translocations of most endemic Burkitt lymphomas occur as a consequence of aberrant V-D-J recombination of variable, diversity, and joining gene segments, catalyzed by the recombinase enzymes. This phenomenon was first noted in follicular lymphomas and chronic lymphocytic leukemias of the B-cell lineage and has been described in T-cell malignancies as well. In each of these cases, analysis of the nucleotide sequence at chromosome breakpoints demonstrated the involvement of immunoglobulin heavy chain JH or T-cell-receptor alpha-chain J alpha gene segments in the translocation. We now have cloned and sequenced both the 8q- and 14q+ translocation breakpoints deriving from the t(8;14) translocation of the endemic Burkitt lymphoma line Daudi. Our data show that the translocation resulted from a reciprocal exchange between the DH region on chromosome 14 and sequences far 5' of the MYC protooncogene on chromosome 8. Features of the nucleotide sequences surrounding the breakpoint further implicate the V-D-J joining machinery in the genesis of chromosome translocations in endemic Burkitt lymphomas and, more generally, in other lymphoid malignancies as well.
对携带t(8;14)染色体易位的伯基特淋巴瘤进行的近期分子分析表明,关于易位发生的分子机制存在二分法。大多数散发性伯基特肿瘤携带的易位显然是由于免疫球蛋白同种型转换过程中的错误而产生的。相比之下,有证据表明,大多数地方性伯基特淋巴瘤的易位是由重组酶催化的可变、多样和连接基因片段的异常V-D-J重组所致。这种现象最初在B细胞系的滤泡性淋巴瘤和慢性淋巴细胞白血病中被注意到,在T细胞恶性肿瘤中也有描述。在这些病例中的每一个中,对染色体断点处核苷酸序列的分析表明免疫球蛋白重链JH或T细胞受体α链Jα基因片段参与了易位。我们现在已经克隆并测序了源自地方性伯基特淋巴瘤细胞系Daudi的t(8;14)易位的8q-和14q+易位断点。我们的数据表明,易位是由14号染色体上的DH区域与8号染色体上MYC原癌基因5'端远处的序列相互交换所致。断点周围核苷酸序列的特征进一步表明V-D-J连接机制参与了地方性伯基特淋巴瘤以及更普遍的其他淋巴样恶性肿瘤中染色体易位的发生。