Imprinting of a RING zinc-finger encoding gene in the mouse chromosome region homologous to the Prader-Willi syndrome genetic region

Imprinting of a RING zinc-finger encoding gene in the mouse chromosome region homologous to the Prader-Willi syndrome genetic region
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DOI:
10.1093/hmg/8.5.795
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发表时间:
1999-05-01
影响因子:
3.5
通讯作者:
Nicholls, RD
Nicholls, RD
中科院分区:
生物学2区
文献类型:
--
作者:
Jong, MTC;Carey, AH;Nicholls, RD

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人类Prader-Willi综合征(PWS)区域的一个新位点编码印迹ZNF 127和反义ZNF 127 AS基因。在此,我们发现小鼠ZNF 127的直系同源物Zfp 127编码一个同源的推定锌指多肽,具有一个RING(C3 HC 4)和三个C3 H锌指结构域,表明其功能为核糖核蛋白。我们发现Zfp 127仅在脑、心脏和肾中由父本等位基因表达。类似地,Zfp 127在源自雄激素胚胎干细胞和正常胚胎的分化细胞中表达,但在源自单性生殖胚胎干细胞的分化细胞中不表达。我们推测,配子印记可能会设置,至少部分,由Zfp 127在前和后减数分裂的雄性生殖细胞的转录活性。因此,Zfp 127是一种新的印迹基因,可能在PWS小鼠模型的印迹表型中发挥作用。
A novel locus in the human Prader-Willi syndrome (PWS) region encodes the imprinted ZNF127 and anti-sense ZNF127AS genes. Here, we show that the mouse ZNF127 ortholog, Zfp127, encodes a homologous putative zinc-finger polypeptide, with a RING (C3HC4) and three C3H zinc-finger domains that suggest function as a ribonucleoprotein, By the use of RT-PCR across an in-frame hexamer tandem repeat and RNA from a Mus musculusxM.spretus F-1 interspecific cross, we show that Zfp127 is expressed only from the paternal allele in brain, heart and kidney. Similarly, Zfp127 is expressed in differentiated cells derived from androgenetic embryonic stem cells and normal embryos but not those from parthogenetic embryonic stem cells. We hypothesize that the gametic imprint may be set, at least in part, by the transcriptional activity of Zfp127 in pre- and post-meiotic male germ cells. Therefore, Zfp127 is a novel imprinted gene that may play a role in the imprinted phenotype of mouse models of PWS.