The Prader-Willi/Angelman imprinted domain and its control center.

Conference Paper

The present review focuses on the recent advances towards understanding the mode of operation of the imprinting center (IC) within the Prader-Willi/Angelman syndromes (PWS/AS) domain. Special emphasis is put on the elucidation of the functional interaction between the two parts of the center, AS-IC and PWS-IC. The recent studies, on which the review is based, reveal cis-acting elements and trans-acting proteins that constitute the two parts of the IC and presumably provide the molecular mechanism for this interaction. AS-IC acquires the primary imprint during gametogenesis by establishing the maternal epigenotype. The unmethylated maternal allele of the AS-IC binds, very likely, a trans-acting factor that confers methylation on the PWS-IC maternal allele after fertilization. It is assumed that the PWS-IC paternal epigenotype, once established, spreads across the entire PWS/AS domain in the soma.

Full Text

Duke Authors

Cited Authors

  • Kantor, B; Shemer, R; Razin, A

Published Date

  • 2006

Published In

Volume / Issue

  • 113 / 1-4

Start / End Page

  • 300 - 305

PubMed ID

  • 16575193

Electronic International Standard Serial Number (EISSN)

  • 1424-859X

Digital Object Identifier (DOI)

  • 10.1159/000090845

Conference Location

  • Switzerland