DBE-T is generated preferentially in FSHD patients, as a consequence of D4Z4 repeat deletions and recruits the Trithorax protein Ash1L, to promote H3K36 dimethylation and transcription of the FSHD locus (Cabianca et al., 2012; Figure 3). This evidence concerns the gene ASH1L and facioscapulohumeral muscular dystrophy.