Larson R C, Osada H, Larson T A, Lavenir I, Rabbitts T H
MRC Laboratory of Molecular Biology, Cambridge, UK.
Oncogene. 1995 Sep 7;11(5):853-62.
RBTN2 is activated by the chromosomal translocation t(11;14) (P13;p11) in some T cell leukaemias. Histologically similar T cell tumours develop with long latency in transgenic mice when either CD2 or thy1.1 promoters control rbtn2 expression. During the asymptomatic period, perturbation of T cell differentiation occurs in the thymus. The major anomalies present during this phase are an increase in the percentage of large thymocytes lacking CD4 and CD8 markers and also of small thymocytes express both the T cell marker CD3 and the B cell-specific form of CD45. These abnormal T cell populations can be clonal and thus a primary result of aberrant expression of the LIM-protein Rbtn2 is alteration of T cell differentiation preceding overt malignancy. These data provide a biological explanation for the role of Rbtn2 in tumorigenesis and presumably RBTN2 expression in T cells after the translocation t(11;14) in children has the same effect.
在某些T细胞白血病中，RBTN2通过染色体易位t(11;14)(P13;p11)被激活。当CD2或thy1.1启动子控制rbtn2表达时，组织学上相似的T细胞肿瘤在转基因小鼠中出现较长潜伏期。在无症状期，胸腺中T细胞分化发生紊乱。此阶段出现的主要异常是缺乏CD4和CD8标记的大胸腺细胞百分比增加，以及同时表达T细胞标记CD3和B细胞特异性形式CD45的小胸腺细胞百分比增加。这些异常T细胞群体可能是克隆性的，因此，LIM蛋白Rbtn2异常表达的主要结果是在明显恶性肿瘤之前T细胞分化发生改变。这些数据为Rbtn2在肿瘤发生中的作用提供了生物学解释，并且推测儿童中t(11;14)易位后T细胞中RBTN2的表达具有相同作用。
