D4Z4 binding element transcript (DBE-T)

D4Z4 binding element transcript (DBE-T) is a long non-coding RNA expressed from the D4Z4 subtelomeric repeats on chromosome 4, particularly upregulated in facioscapulohumeral muscular dystrophy (FSHD) myoblasts[1][2][3]. DBE-T works as a chromatin-associated RNA that recruits the histone methyltransferase Ash1L to D4Z4 repeats, leading to a change in chromatin state and the derepression of nearby genes, including the disease-causing DUX4 gene[1]. This lncRNA thereby functions as an epigenetic regulator, linking chromatin architecture to the transcriptional activator events central to FSHD pathology. DBE-T itself does not encode a protein and is not a direct therapeutic target, but plays a key role in the molecular mechanisms driving FSHD[1][2][3].

Other names

DBE-TDUX4L30chromatin-associated long non-coding RNAD4Z4 binding element transcript (non-protein coding)double homeobox 4 like 4 pseudogeneDBET