不寻常的de novo t(13;15)(q12.1;p13)易位导致复杂的镶嵌现象,包括跳跃易位。

Annales de genetique Pub Date : 1998-01-01
P Petit, K Devriendt, J R Vermeesch, P De Cock, J P Fryns
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引用次数: 0

摘要

我们报告了一例神经感觉性耳聋、白内障和中度智力低下的患者,其主要细胞系由45、XY、-13、-15、+t(13;15)型易位组成。利用荧光原位杂交(FISH)技术和各种条带技术,分别在13q12.1和15p13位点确定了易位的断点。其他5个细胞系存在,但比例较低,其中一个显示t(13;15)串联易位。在Robertsonian易位融合位点和串联易位融合位点均可检测到间隙端粒序列。因此,嵌合现象似乎是染色体不稳定的结果,涉及优势细胞系的t(13;15)融合区,并与间质端粒序列的存在有关。目前的观察结果表明,在中心体周围13q12区域,可能存在一个与神经感觉耳聋有关的基因。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Unusual de novo t(13;15)(q12.1;p13) translocation leading to complex mosaicism including jumping translocation.

We report on a patient with neurosensory deafness, cataract and moderate mental retardation showing a constitutional mosaicism with the predominant cell line consisting of a 45,XY,-13,-15,+t(13;15) translocation of the Robertsonian type. By means of fluorescence in situ hybridization (FISH) using a panel of acrocentric pericentromeric probes and various banding techniques, the breakpoints in the translocation were determined at 13q12.1 and 15p13 respectively. Five other cell lines were present, at low percentage, one of them showing a t(13;15) tandem translocation. Interstitial telomeric sequences could be detected at the translocation fusion sites in both the Robertsonian and tandem translocations. The mosaicism appears therefore to be a consequence of chromosomal instability involving the t(13;15) fusion region of the predominant cell line, and related to the presence of interstitial telomeric sequences. The present observation suggests that in the pericentromeric 13q12 region, a gene involved in neurosensory deafness may be located.

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