Human Molecular Genetics, Vol 8, 795-803, Copyright © 1999 by Oxford University Press
MT Jong, AH Carey, KA Caldwell, MH Lau, MA Handel, DJ Driscoll, CL Stewart, EM Rinchik and RD Nicholls
A novel locus in the human Prader-Willi syndrome (PWS) region encodes the
imprinted ZNF127 and antisense 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 musculus x M.spretus
F1interspecific 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.
ARTICLES
Imprinting of a RING zinc-finger encoding gene in the mouse chromosome region homologous to the Prader-Willi syndrome genetic region
Department of Genetics, Case Western Reserve University School of Medicine and Center for Human Genetics, University Hospitals of Cleveland, 10900 Euclid Avenue, Cleveland, OH 44106-4955, USA.
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