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两例B细胞非霍奇金淋巴瘤中因t(3;13)(q27;q14)染色体易位导致L-原肌球蛋白(LCP1)基因与LAZ3(BCL6)基因发生非随机融合。

Nonrandom fusion of L-plastin(LCP1) and LAZ3(BCL6) genes by t(3;13)(q27;q14) chromosome translocation in two cases of B-cell non-Hodgkin lymphoma.

作者信息

Galiègue-Zouitina S, Quief S, Hildebrand M P, Denis C, Detourmignies L, Laï J L, Kerckaert J P

机构信息

Unité 524 INSERM, Institut de Recherches sur le Cancer de Lille, Lille, France.

出版信息

Genes Chromosomes Cancer. 1999 Oct;26(2):97-105.

Abstract

The LAZ3(BCL6) gene on chromosome band 3q27 is nonrandomly disrupted in B-cell non-Hodgkin lymphoma (B-NHL) by chromosomal translocations clustered within a 3.3-kb MTC (major translocation cluster) located between the two first noncoding exons. These translocations generally result in the expression of a chimeric mRNA transcript between the LAZ3 gene and sequences derived from the partner chromosome. Using RACE RT-PCR, we previously demonstrated fusion of LAZ3 with the RhoH/TTF gene, a hemopoietic cell-specific small GTPase involved in cytoskeleton organization, and with the BOB1/OBF1 gene, a B-cell-specific coactivator of octamer-binding transcription factors, following translocations t(3;4)(q27;p13) and t(3;11)(q27;q23), respectively. Here we report the identification of the L-Plastin(LCP1) gene as a novel LAZ3 partner in chimeric transcripts resulting from a t(3;13)(q27;q14) translocation, in two cases of B-cell lymphoma. As a consequence of the translocation, the 5' regulatory region of each gene was exchanged, creating both LCP1-LAZ3 and reciprocal LAZ3-LCP1 fusion transcripts in one case, and only a LCP1-LAZ3 fusion transcript in the other. The 13q14 chromosome region is frequently disrupted in various proliferative disorders, and the LCP1 gene defines a new breakpoint site in this region. This gene encodes an actin-binding protein and is the second LAZ3 partner gene, with the RhoH/TTF gene, involved in actin cytoskeleton organization. Genes Chromosomes Cancer 26:97-105, 1999.

摘要

位于染色体3q27带上的LAZ3(BCL6)基因在B细胞非霍奇金淋巴瘤(B-NHL)中会因染色体易位而发生非随机破坏,这些易位集中在两个首个非编码外显子之间一个3.3kb的主要易位簇(MTC)内。这些易位通常会导致LAZ3基因与来自伙伴染色体的序列之间产生嵌合mRNA转录本的表达。利用RACE RT-PCR技术,我们之前证实,在分别发生t(3;4)(q27;p13)和t(3;11)(q27;q23)易位后,LAZ3与RhoH/TTF基因(一种参与细胞骨架组织的造血细胞特异性小GTP酶)以及与BOB1/OBF1基因(一种八聚体结合转录因子的B细胞特异性共激活因子)发生了融合。在此,我们报告在两例B细胞淋巴瘤中,鉴定出L-丝动蛋白(LCP1)基因是由t(3;13)(q27;q14)易位产生的嵌合转录本中的一个新的LAZ3伙伴。作为易位的结果,每个基因的5'调控区域发生了交换,在一个病例中产生了LCP1-LAZ3和相互的LAZ3-LCP1融合转录本,而在另一个病例中只产生了LCP1-LAZ3融合转录本。13q14染色体区域在各种增殖性疾病中经常被破坏,LCP1基因在该区域定义了一个新的断点位点。该基因编码一种肌动蛋白结合蛋白,并且是第二个与RhoH/TTF基因一起参与肌动蛋白细胞骨架组织的LAZ3伙伴基因。《基因、染色体与癌症》26:97 - 105,1999年。

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