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| http://dx.doi.org/10.1016/j.jid.2019.03.1141 | DOI Listing |
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HOPX Is a ZNF750 Target that Promotes Late Epidermal Differentiation.
J Invest Dermatol
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Department of Dermatology, University of California-San Diego, La Jolla, California, USA. Electronic address:
Genomic profiling of a human organotypic model of AEC syndrome reveals ZNF750 as an essential downstream target of mutant TP63.
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Program in Epithelial Biology, Stanford University School of Medicine, Stanford, CA 94305, USA.
The basis for impaired differentiation in TP63 mutant ankyloblepharon-ectodermal dysplasia-clefting (AEC) syndrome is unknown. Human epidermis harboring AEC TP63 mutants recapitulated this impairment, along with downregulation of differentiation activators, including HOPX, GRHL3, KLF4, PRDM1, and ZNF750. Gene-set enrichment analysis indicated that disrupted expression of epidermal differentiation programs under the control of ZNF750 and KLF4 accounted for the majority of disrupted epidermal differentiation resulting from AEC mutant TP63.
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