Document Detail

Disruption of the genomic imprint in trans with homologous recombination at Snrpn in ES cells.
MedLine Citation:
PMID:  14666508     Owner:  NLM     Status:  MEDLINE    
In gene targeting studies of the Prader-Willi syndrome (PWS)/Angelman syndrome (AS) domain in mouse ES cells, we recovered only recombinants with the paternal allele for constructs at exons 2 or 3 of the imprinted, maternally silenced Snurf-Snrpn gene. These sites lie close to the imprinting center (IC) for this domain. In contrast, recombinants for Ube3a within the same imprinted domain were recovered with equal frequency on the maternal and paternal alleles. In addition, gene targeting of the paternal allele for Snurf-Snrpn resulted in partial or complete demethylation in trans with activation of expression for the previously silenced maternal allele. The imprint switching of the maternal allele in trans is not readily explained by competition for trans-acting factors and adds to a growing body of evidence indicating homologous association of oppositely imprinted chromatin domains in somatic mammalian cells.
Ting-Fen Tsai; Jan Bressler; Yong-Hui Jiang; Arthur L Beaudet
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Publication Detail:
Type:  Journal Article; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Genesis (New York, N.Y. : 2000)     Volume:  37     ISSN:  1526-954X     ISO Abbreviation:  Genesis     Publication Date:  2003 Dec 
Date Detail:
Created Date:  2003-12-10     Completed Date:  2004-08-25     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  100931242     Medline TA:  Genesis     Country:  United States    
Other Details:
Languages:  eng     Pagination:  151-61     Citation Subset:  IM    
Copyright Information:
Copyright 2003 Wiley-Liss, Inc.
Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas, USA.
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MeSH Terms
Angelman Syndrome / genetics
Base Sequence
DNA Methylation*
Exons / genetics
Genomic Imprinting / genetics*
Mice, Inbred C57BL
Molecular Sequence Data
Nuclear Proteins / genetics*
Polymorphism, Genetic / genetics
Prader-Willi Syndrome / genetics
Recombination, Genetic*
Stem Cells / metabolism
Ubiquitin-Protein Ligases / genetics*
Reg. No./Substance:
0/Nuclear Proteins; 0/SNURF protein, human; 0/SNURF protein, mouse; 0/UBE3A protein, human; EC Ligases

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