Figure 4
From: Long noncoding RNAs, emerging players in muscle differentiation and disease
Role of DBE-T long noncoding RNA (lncRNA) in facioscapulohumeral muscular dystrophy (FSHD). In healthy individuals, the D4Z4 array displays from 11 to more than 100 units and is extensively bound by Polycomb group proteins (PcG), leading to the repression of the locus. In FSHD patients, the reduction of D4Z4 copy number to below 11 units causes decreased PcG binding and hence reduced silencing. This allows the transcription of the lncRNA DBE-T that remains associated to the FSHD locus and recruits the TrxG protein ASHL1 leading to activation of FSHD candidate genes.