Wilkie A O, Yang S P, Summers D, Poole M D, Reardon W, Winter R M
Institute of Molecular Medicine, John Radcliffe Hospital, Headington, Oxford, UK.
J Med Genet. 1995 Mar;32(3):174-80. doi: 10.1136/jmg.32.3.174.
We describe three families segregating different reciprocal chromosome translocations, t(7;18)(p21.2;q23), t(2;7)(q21.1;p21.2), and t(5;7)(p15.3;p21.2). A total of seven apparently balanced carriers have been identified and all manifest features of the Saethre-Chotzen syndrome, although only two have overt craniosynostosis. In one family the carriers are immediately recognisable by their unusual ears, and clefts of the hard or soft palate are present in all three families. These observations extend previous linkage and cytogenetic evidence that a locus for Saethre-Chotzen syndrome resides in band 7p21.2.
我们描述了三个家族,它们分别携带不同的相互染色体易位,即t(7;18)(p21.2;q23)、t(2;7)(q21.1;p21.2)和t(5;7)(p15.3;p21.2)。总共鉴定出7名明显平衡的携带者,他们均表现出塞特雷-乔岑综合征的特征,尽管只有两人有明显的颅缝早闭。在一个家族中,携带者因其异常的耳朵很容易被识别出来,并且在所有三个家族中都存在硬腭或软腭裂。这些观察结果扩展了先前的连锁和细胞遗传学证据,即塞特雷-乔岑综合征的一个基因座位于7p21.2带。