Kousseff B G
Am J Med Genet. 1982 Dec;13(4):431-9. doi: 10.1002/ajmg.1320130412.
A patient with Prader-Labhart-Willi syndrome (PLWS) was found to have mosaic partial trisomy 15: 46,XY/47,XY, + del(15) (pter leads to q1.3:) in both lymphocytes and fibroblasts. Thus, another novel aberration is added to the spectrum of chromosome abnormalities seen in this syndrome. The spectrum includes deletion of the short arm of chromosome 15, interstitial deletion of 15q1.2, inverted duplication of 15p (tetrasomy 15p), partial trisomy 15 different from that encountered in this patient, and a variety of aberrations involving other chromosomes. A hypothesis that the chromosome aberrations are due to a presumed gene for the PLWS may have merit and could be tested in the laboratory by exposing chromosomes of patients with PLWS to mutagens to search for secondary chromosome derangements.
一名普拉德-威利综合征(PLWS)患者的淋巴细胞和成纤维细胞中均发现有嵌合型部分15号染色体三体:46,XY/47,XY, + del(15) (pter 至 q1.3 :)。因此,在该综合征所见的染色体异常谱中又增加了一种新的畸变。该异常谱包括15号染色体短臂缺失、15q1.2间质性缺失、15号染色体短臂反向重复(15号染色体短臂四体)、与该患者不同的15号染色体部分三体,以及涉及其他染色体的多种畸变。关于染色体畸变是由假定的普拉德-威利综合征基因所致的假说可能有价值,并且可以在实验室中通过将普拉德-威利综合征患者的染色体暴露于诱变剂来寻找继发性染色体紊乱进行验证。