Sj. Chamberlain et Ci. Brannan, The Prader-Willi syndrome imprinting center activates the paternally expressed murine Ube3a antisense transcript but represses paternal Ube3a, GENOMICS, 73(3), 2001, pp. 316-322
The imprinted UBE3A gene exhibits maternal-only expression in specific cell
types in the brain, but exhibits biallelic expression in other cell types.
UBE3A is located adjacent to a cluster of imprinted, paternally expressed
genes that are known to be positively regulated by the Prader-Willi syndrom
e imprinting center (PWS-IC). Here, we examined the effect of the PWS-IC on
the UBE3A locus. Using intersubspecific crosses, we found that deletion of
the PWS-IC causes an upregulation of the paternal Ube3a allele. This indic
ates that unlike its positive effect on all the other paternally expressed
transcripts in the region, the PWS-IC negatively regulates the levels of pa
ternal UBE3A. Interestingly, we found that like the human UBE3A locus, the
murine Ube3a locus includes an imprinted, paternally expressed antisense tr
anscript. We show that this paternal antisense transcript is positively reg
ulated by the PWS-IC. These results are consistent with a model in which th
e PWS-IC mediates activation and maintenance of paternal gene expression in
the 15q11-q13 region, with repression of the paternal UBE3A gene occurring
as an indirect result of expression of the antisense transcript, (C) 2001
Academic Press.