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

被引:99
作者
Jong, MTC
Carey, AH
Caldwell, KA
Lau, MH
Handel, MA
Driscoll, DJ
Stewart, CL
Rinchik, EM
Nicholls, RD
机构
[1] Case Western Reserve Univ, Sch Med, Dept Genet, Cleveland, OH 44106 USA
[2] Univ Hosp Cleveland, Ctr Human Genet, Cleveland, OH 44106 USA
[3] Univ Florida, Coll Med, Div Pediat Genet, RC Philips Unit, Gainesville, FL 32610 USA
[4] Univ Florida, Coll Med, Ctr Mammalian Genet, Gainesville, FL 32610 USA
[5] Roche Inst Mol Biol, Nutley, NJ 07110 USA
[6] Univ Tennessee, Dept Biochem & Cellular & Mol Biol, Knoxville, TN 37996 USA
[7] Oak Ridge Natl Lab, Div Life Sci, Oak Ridge, TN 37831 USA
关键词
D O I
10.1093/hmg/8.5.795
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
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.
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收藏
页码:795 / 803
页数:9
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