biostudies-literatureDuBose AJNICHD NIH HHS3446-50https://www.ebi.ac.uk/biostudies/studies/S-EPMC3295271biostudies-literatureUnknown109(9)Imprinted gene expression associated with Prader-Willi syndrome (PWS) and Angelman syndrome (AS) is controlled by two imprinting centers (ICs), the PWS-IC and the AS-IC. The PWS-IC operates in cis to activate transcription of genes that are expressed exclusively from the paternal allele. We have created a conditional allele of the PWS-IC to investigate its developmental activity. Deletion of the paternal PWS-IC in the embryo before implantation abolishes expression of the paternal-only genes in the neonatal brain. Surprisingly, deletion of the PWS-IC in early brain progenitors does not affect the subsequent imprinted status of PWS/AS genes in the newborn brain. These results indicate that the PWS-IC functions to protect the paternal epigenotype at the epiblast stage of development but is dispensable thereafter.Proceedings of the National Academy of Sciences of the United States of AmericaTemporal and developmental requirements for the Prader-Willi imprinting center.PMC3295271R01 HD 037872R01 HD037872Resnick JLDuBose AJSmith EYJohnstone KAfalseTemporal and developmental requirements for the Prader-Willi imprinting center.Imprinted gene expression associated with Prader-Willi syndrome (PWS) and Angelman syndrome (AS) is controlled by two imprinting centers (ICs), the PWS-IC and the AS-IC. The PWS-IC operates in cis to activate transcription of genes that are expressed exclusively from the paternal allele. We have created a conditional allele of the PWS-IC to investigate its developmental activity. Deletion of the paternal PWS-IC in the embryo before implantation abolishes expression of the paternal-only genes in the neonatal brain. Surprisingly, deletion of the PWS-IC in early brain progenitors does not affect the subsequent imprinted status of PWS/AS genes in the newborn brain. These results indicate that the PWS-IC functions to protect the paternal epigenotype at the epiblast stage of development but is dispensable thereafter.2012-01-01T00:00:00Z2012 Feb2025-04-29T09:57:28.698Z2019-03-27T00:50:54ZS-EPMC32952712233191010.1073/pnas.1115057109