The orthodenticle homeobox 2, encoded by OTX2 gene, is a key transcription factor in developmental processes. In particular, it is required for the early specification of the brain and the embryonic development of sensory organs, including the, pineal gland, pituitary gland, inner part of the ear, eyes, and optic nerve. In later stages, it is important for maintaining intact retina and brain function. In addition, it acts as a transcriptional repressor and a gatekeeper of myogenic and neuronal differentiation in medulloblastoma cells. OTX2 binds to the MyoD1 core enhancer through its homeobox domain and the remarkable repressor activity exhibited by the homeobox domain renders OTX2 transcriptionally repressive.
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