Chronic myeloid leukemia may be associated with several bcr-abl transcripts including the acute lymphoid leukemia-type 7 kb transcript.
Chronic myeloid leukemia may be associated with several bcr-abl transcripts including the acute lymphoid leukemia-type 7 kb transcript.
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DOI：
10.1182/blood.v75.5.1146.1146
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发表时间：
1990-03
期刊：
影响因子：
20.3
通讯作者：
L. Selleri;M. Lindern;A. Hermans;D. Meijer;G. Torelli;G. Grosveld
中科院分区：
文献类型：
--
作者：
L. Selleri;M. Lindern;A. Hermans;D. Meijer;G. Torelli;G. Grosveld
在大多数费城 (Ph) 阳性慢性粒细胞白血病 (CML) 患者中，c-abl 基因与 bcr 基因融合，导致 8.5 kb 嵌合 bcr-abl mRNA 转录，该 mRNA 被翻译为 p210bcr-abl 融合蛋白。在大约 50% 的 Ph 阳性急性淋巴细胞白血病 (ALL) 中，bcr-abl 基因融合与 CML 相同，而在 50% 的 Ph 阳性急性淋巴细胞白血病 (ALL) 中，这两个基因之间发生替代融合，其中中央 bcr 序列缺失。这导致 7 kb bcr-abl mRNA 的转录，编码 P190bcr-abl 融合蛋白。对两名慢性期 Ph 阳性 CML 患者的 bcr-abl cDNA 嵌合部分进行克隆和测序表明，一名患者与 Ph 阳性 ALL 一样，所有中心 bcr 序列均缺失，而另一名患者则部分 bcr 中心序列被删除。因此，我们推测其中一名患者中存在 7 kb 嵌合 ALL 型 mRNA 不足以驱动该病例的急性而非慢性白血病过程。此处描述的中心 bcr 序列的删除定义了迄今为止 CML 患者中 bcr-abl 融合基因的最低序列要求。
In the majority of Philadelphia (Ph)-positive chronic myeloid leukemia (CML) patients, the c-abl gene is fused to the bcr gene, resulting in the transcription of an 8.5 kb chimeric bcr-abl mRNA, which is translated into a p210bcr-abl fusion protein. In about 50% of the Ph-positive acute lymphoid leukemias (ALL), the bcr-abl gene fusion is identical to CML, while in 50% an alternative fusion between these two genes occurs, in which the central bcr-sequences are absent. This results in transcription of a 7 kb bcr-abl mRNA, encoding a P190bcr-abl fusion protein. Cloning and sequencing of the chimeric part of bcr-abl cDNAs from two Ph-positive CML patients in chronic phase showed that in one patient, as in the Ph-positive ALL, all central bcr sequences are absent, while in the other patient, part of the bcr central sequences are deleted. Therefore, we speculate that the presence of the 7 kb chimeric ALL type mRNA in one of the patients is not sufficient to drive an acute rather than a chronic leukemic process in this case. The deletions of the central bcr-sequences described here define the minimal sequence requirement of the bcr-abl fusion gene in CML patients so far.