UTF1 is a 341 amino acid protein that localizes to the nucleus and is subject to post-translational phosphorylation. Associating with the TFIID complex via an interaction with the TATA box binding protein (TFIID), UTF1 binds to the N-terminal region of ATF-2 and, via this binding, acts as a transcriptional coactivator of ATF-2, thereby enhancing transcriptional activity. Human UTF1 shares 64% homology with its mouse counterpart, suggesting a similar role between species. The gene encoding UTF1 maps to human chromosome 10, which houses over 1,200 genes and comprises nearly 4.5% of the human genome. Defects in some of the genes that map to chromosome 10 are associated with Charcot-Marie Tooth disease, Jackson-Weiss syndrome, Usher syndrome, nonsyndromatic deafness, Wolman’s syndrome, Cowden syndrome, multiple endocrine neoplasia type 2 and porphyria.