Kurzrock R, Kloetzer W S, Talpaz M, Blick M, Walters R, Arlinghaus R B, Gutterman J U
Blood. 1987 Jul;70(1):233-6.
The aberrant abl protein product of a chronic myelogenous leukemia (CML) blast crisis cell line (K562) and of five Philadelphia chromosome-positive CML patients in blast crisis were analyzed by an immune complex kinase assay using two antipeptide sera generated against the hydrophilic domain of v-abl and a region within the third exon of the breakpoint cluster region (bcr) respectively. Both the anti-abl and anti-bcr sera detected a 210 kd band in extracts derived from K562 cells and from two CML patients with myeloid blast crisis. p210 was detected by the anti-abl but not the anti-bcr sera in three CML patients with myeloid (one patient) and lymphoid (two patients) blast crisis, indicating the absence of bcr exon 3 in this protein. Southern blot analysis on DNA derived from one of the patients in the latter group was consistent with the break on chromosome 22 occurring 5' to bcr exon 3. Our observations demonstrate that the Philadelphia translocation results in the generation of a chimeric bcr-abl protein with at least two molecular variants, both of which are enzymatically active as protein kinases.
利用分别针对v-abl亲水区和断裂簇区域（bcr）第三外显子内一个区域产生的两种抗肽血清，通过免疫复合物激酶分析，对慢性髓性白血病（CML）急变期细胞系（K562）以及5例费城染色体阳性的CML急变期患者的异常abl蛋白产物进行了分析。抗abl血清和抗bcr血清在源自K562细胞以及两名髓系急变期CML患者的提取物中均检测到一条210kd的条带。在3例髓系（1例患者）和淋巴系（2例患者）急变期的CML患者中，抗abl血清检测到了p210，但抗bcr血清未检测到，这表明该蛋白中不存在bcr外显子3。对后一组中一名患者的DNA进行的Southern印迹分析与22号染色体上的断裂发生在bcr外显子3的5'端这一结果一致。我们的观察结果表明，费城易位导致产生了一种嵌合的bcr-abl蛋白，该蛋白至少有两种分子变体，二者均具有蛋白激酶的酶活性。
