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白血病与发育过程中的LIM结构域蛋白

LIM domain proteins in leukaemia and development.

作者信息

Sánchez-García I, Rabbitts T H

机构信息

MRC Laboratory of Molecular Biology, Cambridge, UK.

出版信息

Semin Cancer Biol. 1993 Dec;4(6):349-58.

PMID:8142620
Abstract

T cell acute leukaemias involve a number of different classes of oncogenes. A group of such genes is the RBTN family located on chromosomes 11 and 12. Two members of this family, RBTN1/Ttg-1 and RBTN2/Ttg-2, are located near recurring T cell acute lymphocytic leukaemia-associated translocations. Chromosomal translocations to both RBTN1/Ttg-1 and RBTN2/Ttg-2 involve T cell receptor (TCR) genes as result of an erroneous V(D)J joining process. RBTN1/Ttg-1 and RBTN2/Ttg-2 encode related proteins consisting of two cysteine-rich regions called LIM domains. The fact that LIM domains can be found with or without associated homeodomain led to the suggestion that the LIM domains may function as regulators of transcription, and that alterations of transcription networks, after chromosomal translocations, lead to leukaemia. This is a common feature that has been noted in the activation of transcription factors with a variety of structural motifs that include the basic helix-loop-helix motif and the homeodomain in leukaemias.

摘要

T细胞急性白血病涉及多种不同类型的癌基因。这类基因中的一组是位于11号和12号染色体上的RBTN家族。该家族的两个成员,RBTN1/Ttg-1和RBTN2/Ttg-2,位于复发性T细胞急性淋巴细胞白血病相关易位附近。由于错误的V(D)J连接过程,向RBTN1/Ttg-1和RBTN2/Ttg-2的染色体易位都涉及T细胞受体(TCR)基因。RBTN1/Ttg-1和RBTN2/Ttg-2编码由两个称为LIM结构域的富含半胱氨酸区域组成的相关蛋白质。LIM结构域可与或不与相关的同源结构域一起出现,这一事实表明LIM结构域可能作为转录调节因子发挥作用,并且染色体易位后转录网络的改变会导致白血病。这是在白血病中具有多种结构基序(包括基本螺旋-环-螺旋基序和同源结构域)的转录因子激活过程中所观察到的一个共同特征。

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