Daniel A
Hum Genet. 1979 Oct 1;51(2):171-82. doi: 10.1007/BF00287173.
Interchange segment sizes and the sizes of chromosome imbalance arising from the different modes of meiotic segregation were measured in a selected sample of 20 reciprocal translocations (Rep). The Rep were selected by two modes of ascertainment: (I) neonates with an unbalanced form of the translocation, and (II) couples with recurrent spontaneous abortions without evidence of full-term translocation aneuploid offspring. The measurements (% of haploid autosomal length: %HAL) were plotted as the observed or potential chromosomal imbalance with monosomy (abscissa) and trisomy (ordinate). It was found that (a) the interchange segments were larger in the spontaneous abortion Rcp, (b) that all of the imbalances observed in full-term neonates plotted close to the origin and to the left of the line joining 4% trisomy to 2% monosomy, and (c) the imbalances observed in the neonates in each individual Rcp were of the smallest size possible arising by any segregation mode. It was concluded that a major factor in the survival to term of aneuploid conceptuses is the size (proportion of genome) of the chromosome abnormality, irrespective of the origin of the chromosome regions. These results are discussed in relation to their use as a model to evaluate the risk of abnormal offspring in the progeny of translocation heterozygotes (the Chromosome Imbalance Size-Viability Model).
在20个相互易位(Rep)的选定样本中,测量了互换片段大小以及不同减数分裂分离模式产生的染色体不平衡大小。这些相互易位是通过两种确定方式选取的:(I)具有不平衡易位形式的新生儿,以及(II)有反复自然流产且无足月易位非整倍体后代证据的夫妇。测量值(单倍体常染色体长度的百分比:%HAL)被绘制为观察到的或潜在的染色体不平衡,横坐标为单体型,纵坐标为三体型。结果发现:(a)自然流产的相互易位中互换片段更大;(b)足月新生儿中观察到的所有不平衡都靠近原点且位于连接4%三体到2%单体的直线左侧;(c)每个个体相互易位的新生儿中观察到的不平衡是任何分离模式可能产生的最小大小。得出的结论是,非整倍体胚胎存活至足月的一个主要因素是染色体异常的大小(基因组比例),而与染色体区域的来源无关。结合将这些结果用作模型来评估易位杂合子后代中异常后代风险(染色体不平衡大小-生存力模型)对这些结果进行了讨论。