Double homeobox 4 protein (DUX4) is a transcription factor consisted of two closely spaced homeodomains (HD1 and HD2) at the N-terminal region that have sequence-specific binding with DNA, and a transactivation domain at the C-terminal region. DUX4 is expressed during early embryogenesis, regulates genes associated with pre- and post-implantation development. DUX4 expression is epigenetically silenced in most adult tissues except in testis. Misexpression of DUX4 can cause facioscapulohumeral muscular dystrophy (FSHD), and translocated DUX6 overexpression can lead to B cell leukemia.
The 3D model shows the N-terminus (residues 15-155) of transcription factor DUX4 including two homeodomains (HD1and HD2), bound to the double-stranded DNA including consensus sequence 5’-TAATCTAATCA-3’.
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