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Publication : Temporal and developmental requirements for the Prader-Willi imprinting center.

First Author  DuBose AJ Year  2012
Journal  Proc Natl Acad Sci U S A Volume  109
Issue  9 Pages  3446-50
PubMed ID  22331910 Mgi Jnum  J:182162
Mgi Id  MGI:5314849 Doi  10.1073/pnas.1115057109
Citation  DuBose AJ, et al. (2012) Temporal and developmental requirements for the Prader-Willi imprinting center. Proc Natl Acad Sci U S A 109(9):3446-50
abstractText  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.
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