{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,4,20]],"date-time":"2025-04-20T20:24:55Z","timestamp":1745180695527},"reference-count":37,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2011,4,25]],"date-time":"2011-04-25T00:00:00Z","timestamp":1303689600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Journal Cellular Physiology"],"published-print":{"date-parts":[[2011,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The homeodomain transcription factors play crucial roles in many developmental processes ranging from organization of the body plan to differentiation of individual tissues. The homeodomain protein Distal\u2010less\u20103 (DLX3) has an essential role in epidermal stratification and development of ectodermal appendages, placenta and bones. A four\u2010nucleotide deletion in the human DLX3 gene is etiologic for the human hereditary tricho\u2010dento\u2010osseous (TDO) ectodermal dysplasia, a dominant syndrome characterized by abnormalities in hair, nails, teeth, and bones. We have previously demonstrated that DLX3 gene expression induces degradation of \u0394Np63\u03b1, a specific product of the TP63 gene, a master regulator of multi\u2010layered epithelia. Here we show that the DLX3<jats:sup>TDO<\/jats:sup> mutant protein is unable to promote \u0394Np63\u03b1 protein degradation and impairs the expression of cell cycle regulatory proteins and skin differentiation markers. However, we found that in cell expressing equal amounts of mutant and wild\u2010type DLX3, \u0394Np63\u03b1 protein level is efficiently regulated implying that genetic heterozygosity at the DLX3 locus protects TDO patients from developing severe p63\u2010associated skin defects. J. Cell. Physiol. 226: 2189\u20132197, 2011. \u00a9 2010 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/jcp.22553","type":"journal-article","created":{"date-parts":[[2010,12,6]],"date-time":"2010-12-06T14:04:34Z","timestamp":1291644274000},"page":"2189-2197","source":"Crossref","is-referenced-by-count":13,"title":["A dominant mutation etiologic for human tricho\u2010dento\u2010osseous syndrome impairs the ability of DLX3 to downregulate \u0394Np63\u03b1"],"prefix":"10.1002","volume":"226","author":[{"given":"Antonella","family":"Di 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