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

A novel locus in the human Prader-Willi syndrome (PWS) region encodes the i mprinted ZNF127 and anti-sense ZNF127AS genes. Here, we show that the mouse ZNF127 ortholog, Zfp127, encodes a homologous putative zinc-finger polypep tide, with a RING (C3HC4) and three C3H zinc-finger domains that suggest fu nction as a ribonucleoprotein, By the use of RT-PCR across an in-frame hexa mer tandem repeat and RNA from a Mus musculusxM.spretus F-1 interspecific c ross, we show that Zfp127 is expressed only from the paternal allele in bra in, heart and kidney. Similarly, Zfp127 is expressed in differentiated cell s derived from androgenetic embryonic stem cells and normal embryos but not those from parthogenetic embryonic stem cells. We hypothesize that the gam etic 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 mou se models of PWS.