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与特雷彻·柯林斯综合征发病机制相关基因的定位克隆。特雷彻·柯林斯综合征协作组

Positional cloning of a gene involved in the pathogenesis of Treacher Collins syndrome. The Treacher Collins Syndrome Collaborative Group.

出版信息

Nat Genet. 1996 Feb;12(2):130-6. doi: 10.1038/ng0296-130.

Abstract

Treacher Collins syndrome is an autosomal dominant disorder of craniofacial development, which has been localized to chromosome 5q32-33.1. In the present study, the isolation of new polymorphic markers has allowed the identification of overlapping recombination events in two affected individuals. Extension of the transcription map of the critical region proximally has resulted in the isolation of a new gene (which has been named Treacle) of unknown function. The identification of different mutations in five unrelated families, all of which would result in premature termination of the predicted protein, indicates that the Treacher Collins syndrome gene has been positionally cloned.

摘要

特雷彻·柯林斯综合征是一种常染色体显性颅面发育障碍疾病,其致病基因已定位到5号染色体的5q32 - 33.1区域。在本研究中,新的多态性标记的分离使得在两名患病个体中鉴定出了重叠的重组事件。关键区域转录图谱向近端的扩展导致了一个功能未知的新基因(已命名为Treacle)的分离。在五个无关家族中鉴定出不同的突变,所有这些突变都会导致预测蛋白质的过早终止,这表明特雷彻·柯林斯综合征基因已通过定位克隆得到。

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