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Nucleic Acids Research 2004 32(11):3480-3492; doi:10.1093/nar/gkh670
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Published online 29 June 2004

Nucleic Acids Research, Vol. 32 No. 11 © Oxford University Press 2004; all rights reserved

Regulation of the large (~1000 kb) imprinted murine Ube3a antisense transcript by alternative exons upstream of Snurf/Snrpn

Miguel Landers, Daria L. Bancescu, Elodie Le Meur1, Claire Rougeulle2, Heather Glatt-Deeley, Camilynn Brannan3, Françoise Muscatelli1 and Marc Lalande*

Department of Genetics and Developmental Biology, University of Connecticut Health Center, Farmington, CT 06030, USA, 1 NMDA/IBDM, Campus de Luminy Case 907 13288 Marseille Cedex 09, France, 2 Génétique moléculaire murine CNRS URA 1947, Institut Pasteur, 25 rue du Docteur Roux, Paris Cedex 15, 75724, France and 3 Department of Molecular Genetics and Microbiology, University of Florida College of Medicine, Box 100266, Gainesville, FL 32610, USA

* To whom correspondence should be addressed. Tel: +1 860 679 8349; Fax: +1 860 679 8345; Email: lalande{at}uchc.edu

Received April 6, 2004; Revised May 19, 2004; Accepted June 7, 2004

Most cases of Angelman syndrome (AS) result from loss or inactivation of ubiquitin protein ligase 3A (UBE3A), a gene displaying maternal-specific expression in brain. Epigenetic silencing of the paternal UBE3A allele in brain appears to be mediated by a non-coding UBE3A antisense (UBE3A-ATS). In human, UBE3A-ATS extends ~450 kb to UBE3A from the small nuclear ribonucleoprotein N (SNURF/SNRPN) promoter region that contains a cis-acting imprinting center (IC). The concept of a single large antisense transcript is difficult to reconcile with the observation that SNURF/SNRPN shows a ubiquitous pattern of expression while the more distal part of UBE3A-ATS, which overlaps UBE3A, is brain specific. To address this problem, we examined murine transcripts initiating from several alternative exons dispersed within a 500 kb region upstream of Snurf/Snrpn. Similar to Ube3a-ATS, these upstream (U) exon-containing transcripts are expressed at neuronal stages of differentiation in a cell culture model of neurogenesis. These findings suggest the novel hypothesis that brain-specific transcription of Ube3a-ATS is regulated by the U exons rather than Snurf/Snrpn exon 1 as previously suggested from human studies. In support of this hypothesis, we describe U-Ube3a-ATS transcripts where U exons are spliced to Ube3a-ATS with the exclusion of Snurf-Snrpn. We also show that the murine U exons have arisen by genomic duplication of segments that include elements of the IC, suggesting that the brain specific silencing of Ube3a is due to multiple alternatively spliced IC-Ube3a-ATS transcripts.


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