Acar H, Cora T, Erkul I
Department of Medical Genetics, Selçuk University, Medical Faculty, Konya-Türkiye.
Genet Couns. 1999;10(2):163-70.
In this study, we report conventional and molecular cytogenetic studies in a patient with multiple anomalies who is a carrier of a pericentric inversion on chromosome Y and a chromosome 15p+. His parents were phenotypically normal. The father is a carrier of a pericentric inversion of chromosome Y, and the mother carries a large chromosome 15p+ variant. The inverted Y chromosome was demonstrated by GTG- and CBG-banding, and DAPI-staining. The presence of extra chromosomal material on the chromosome 15p, that was C-band and DAPI positive, was demonstrated by trypsin G-banding. This suggests that the extra chromosomal material contained repetitive DNA sequences. NOR-staining indicated the presence a nuclear organizer region at the junction of the chromosome 15p+ material. Fluorescence in situ hybridization (FISH), with chromosome X and Y painting probes, alpha- and classic-satellite probes specific for chromosome Y, alpha- and beta-satellite III probes for chromosome 15 were used to elucidate the nature of both the inverted Y chromosome and chromosome 15p+. The result with chromosome X and Y painting probes, alpha-satellite, classic-satellite, and DYS59 probes specific for chromosome Y revealed the rearrangement of the Y chromosome was an inv(Y)(p11.2q11.22 or q11.23). FISH with alpha-satellite and beta-satellite III probes for chromosome 15 demonstrated that the extra chromosomal material on the chromosome 15 probably represents beta-satellite III sequences. The possible roles of the simultaneous occurrence of an inverted Y and the amplified DNA sequence on chromosome 15p in the abnormal phenotype of the proband are discussed.
在本研究中,我们报告了一名患有多种异常的患者的传统和分子细胞遗传学研究情况,该患者是Y染色体臂间倒位和15号染色体p+的携带者。他的父母表型正常。父亲是Y染色体臂间倒位的携带者,母亲携带一个大的15号染色体p+变异。通过GTG和CBG显带以及DAPI染色证实了倒位的Y染色体。通过胰蛋白酶G显带证实了15号染色体p上存在额外的染色体物质,该物质C带和DAPI呈阳性。这表明额外的染色体物质包含重复DNA序列。NOR染色表明在15号染色体p+物质的连接处存在一个核仁组织区。使用染色体X和Y涂染探针、针对Y染色体的α卫星和经典卫星探针、针对15号染色体的α和β卫星III探针进行荧光原位杂交(FISH),以阐明倒位的Y染色体和15号染色体p+的性质。使用染色体X和Y涂染探针、针对Y染色体的α卫星、经典卫星和DYS59探针的结果显示,Y染色体的重排为inv(Y)(p11.2q11.22或q11.23)。使用针对15号染色体的α卫星和β卫星III探针进行FISH表明,15号染色体上额外的染色体物质可能代表β卫星III序列。讨论了Y染色体倒位和15号染色体p上DNA序列扩增同时出现可能在先证者异常表型中所起的作用。