Fig. 5.

UBE3A-ATS is only expressed in neuron cultures and correlates with paternal UBE3A repression. (A) Map of the SNURF-SNRPN and UBE3A transcripts. White and black oval-shaped circles indicate the unmethylated and methylated PWS-IC, respectively. The solid line with arrows indicates transcripts expressed in iPSCs, whereas the dotted line indicates neural-specific transcripts. AS iPSCs and their neural derivatives lack the entire maternally inherited allele of this region. (B) Northern blot using total RNA from AS and normal iPSCs (I) and iPSC-derived 10-wk-old neurons (N) shows expression of the snoRNAs SNORD116 and SNORD115 during in vitro neural development. (C) RT-PCR using primers spanning multiple exons of SNORD116, SNORD115, and UBE3A-ATS in iPSCs (I), 3-wk-old iPSC-derived neural precursor cells (P), and 10-wk-old iPSC-derived neuron cultures (N).