Mowery-Rushton P A, Driscoll D J, Nicholls R D, Locker J, Surti U
Department of Genetics, Magee Womens Hospital, Pittsburgh, Pennsylvania, 1521 USA.
Am J Med Genet. 1996 Jan 11;61(2):140-6. doi: 10.1002/(SICI)1096-8628(19960111)61:2<140::AID-AJMG7>3.0.CO;2-0.
In order to further our understanding of the epigenetic modifications of DNA and its role in imprinting, we examined DNA methylation patterns of human tissues of uniparental origin. We used complete hydatidiform moles (CHM), which are totally androgenetic conceptions, to examine the paternal methylation pattern in the absence of a maternal contribution and we used ovarian teratomas to represent the maternal counterpart. We carried out an analysis of DNA methylation of a gene which has been shown to contain sites which are differentially methylated in a parent-specific fashion. The gene, ZNF127, is located on chromosome 15q11-q13 in the region associated with Prader-Willi and Angelman syndromes. The parent-of-origin DNA methylation has been postulated to reflect the presence of an imprint and recent studies have confirmed that ZNF127 is differentially expressed only from the paternal chromosome. We identified a unique pattern of hyper- and hypomethylated sites in androgenetic conceptions which was nearly identical to the paternal pattern found in sperm. This may represent the paternal germ-line methylation imprint. We also studied partial hydatidiform moles, non-molar triploid conceptions, normal chorionic villi, and somatic tissue. These all demonstrated a modified DNA methylation pattern characteristic of normal chorionic villi with only limited findings of the imprint. Our results suggest that human androgenetic conceptions may provide an excellent model to analyze epigenetic DNA modifications, such as methylation, in imprinted genes. The paternal allele-specific methylation imprint will also be useful clinically to confirm the androgenetic nature of suspected molar conceptions in which parental blood samples may not be available.
为了进一步了解DNA的表观遗传修饰及其在印记中的作用,我们研究了单亲来源的人体组织的DNA甲基化模式。我们使用完全性葡萄胎(CHM),其完全是由父系遗传的受精卵,来研究在没有母系贡献的情况下的父系甲基化模式,并使用卵巢畸胎瘤来代表母系对应物。我们对一个基因进行了DNA甲基化分析,该基因已被证明含有以亲本特异性方式差异甲基化的位点。ZNF127基因位于15号染色体q11 - q13区域,与普拉德-威利综合征和天使综合征相关。亲本来源的DNA甲基化被假定反映印记的存在,最近的研究证实ZNF127仅从父系染色体上差异表达。我们在父系遗传的受精卵中鉴定出一种独特的高甲基化和低甲基化位点模式,与精子中发现的父系模式几乎相同。这可能代表父系生殖系甲基化印记。我们还研究了部分性葡萄胎、非葡萄胎三倍体受精卵、正常绒毛膜绒毛和体细胞组织。这些都显示出正常绒毛膜绒毛特有的修饰DNA甲基化模式,只有有限的印记发现。我们的结果表明,人类父系遗传的受精卵可能为分析印记基因中的表观遗传DNA修饰,如甲基化,提供一个极好的模型。父系等位基因特异性甲基化印记在临床上也将有助于确认可疑葡萄胎受精卵的父系遗传性质,而此时可能无法获得父母的血样。