Mason D Y, Pulford K A, Bischof D, Kuefer M U, Butler L H, Lamant L, Delsol G, Morris S W
Leukemia Research Fund Immunodiagnostics Unit, John Radcliffe Hospital, Oxford, United Kingdom.
Cancer Res. 1998 Mar 1;58(5):1057-62.
The (2;5)(p23;q35) lymphoma-associated chromosomal translocation creates a novel fusion gene that incorporates parts of the anaplastic lymphoma kinase (ALK) receptor tyrosine kinase and nucleophosmin genes. We report here that the product of this fusion gene accumulates within the nucleoli of neoplastic cells, and that previous reports of a predominantly cytoplasmic localization for the protein represent a tissue-processing artifact. However, nucleolar accumulation of nucleophosmin-ALK may not be necessary for its oncogenic action, because an ALK protein expressed in a lymphoma carrying a variant (1;2) chromosomal translocation did not accumulate in nucleoli. Furthermore, an engineered hybrid TPR-ALK protein can transform rodent fibroblasts and produce lymphomas in mice while remaining confined to the cytoplasm. We propose that the transforming action of ALK may not be reliant on its nucleolar localization, a hypothesis that may have implications for studies of other proteins involved in oncogenesis that are relocalized after the creation of fusion genes.
(2;5)(p23;q35)淋巴瘤相关的染色体易位产生了一个新的融合基因,该基因整合了间变性淋巴瘤激酶(ALK)受体酪氨酸激酶和核仁素基因的部分片段。我们在此报告,该融合基因的产物在肿瘤细胞的核仁内积聚,并且先前关于该蛋白主要定位于细胞质的报道是一种组织处理假象。然而,核仁素-ALK在核仁中的积聚对于其致癌作用可能并非必需,因为在携带变异(1;2)染色体易位的淋巴瘤中表达的ALK蛋白并未在核仁中积聚。此外,一种工程化的杂交TPR-ALK蛋白可以转化啮齿动物成纤维细胞并在小鼠中产生淋巴瘤,同时仍局限于细胞质中。我们提出,ALK的转化作用可能不依赖于其核仁定位,这一假设可能对其他参与肿瘤发生的蛋白质的研究具有启示意义,这些蛋白质在融合基因产生后会重新定位。