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

Abstract:

: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.

authors

DuBose AJ,Smith EY,Johnstone KA,Resnick JL

doi

10.1073/pnas.1115057109

subject

Has Abstract

pub_date

2012-02-28 00:00:00

pages

3446-50

issue

9

eissn

0027-8424

issn

1091-6490

pii

1115057109

journal_volume

109

pub_type

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