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First published online December 12, 2006
doi: 10.1242/10.1242/dev.02703

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Homeobox gene Dlx3 is regulated by p63 during ectoderm development: relevance in the pathogenesis of ectodermal dysplasias

Nadezda Radoja¹, Luisa Guerrini², Nadia Lo Iacono^2,3, Giorgio R. Merlo³, Antonio Costanzo⁴, Wendy C. Weinberg⁵, Girolama La Mantia⁶, Viola Calabrò^6,* and Maria I. Morasso^1,*,

¹ Developmental Skin Biology Unit, NIAMS, NIH, Bethesda, MD 20892, USA.
² Department of Biomolecular and Biotechnological Sciences, University of Milan, Via Celoria 26. 20133, Milan, Italy.
³ Dulbecco Telethon Institute c/o Istituto Tecnologie Biomediche CNR, 20100 Milan, Italy.
⁴ Department of Dermatology, University of Rome `Tor Vergata', Viale Oxford 81. 00133 Rome, Italy.
⁵ Division of Monoclonal Antibodies, CDER/FDA, Bethesda, MD 20892, USA.
⁶ Department of Structural and Functional Biology, University of Naples, Via Cinzia 26. 80126 Naples, Italy.

Author for correspondence (e-mail: morassom{at}mail.nih.gov)

Accepted 18 October 2006

SUMMARY

Ectodermal dysplasias (EDs) are a group of human pathological conditions characterized by anomalies in organs derived from epithelial-mesenchymal interactions during development. Dlx3 and p63 act as part of the transcriptional regulatory pathways relevant in ectoderm derivatives, and autosomal mutations in either of these genes are associated with human EDs. However, the functional relationship between both proteins is unknown. Here, we demonstrate that Dlx3 is a downstream target of p63. Moreover, we show that transcription of Dlx3 is abrogated by mutations in the sterile -motif (SAM) domain of p63 that are associated with ankyloblepharon-ectodermal dysplasia-clefting (AEC) dysplasias, but not by mutations found in ectrodactylyectodermal dysplasia-cleft lip/palate (EEC), Limb-mammary syndrome (LMS) and split hand-foot malformation (SHFM) dysplasias. Our results unravel aspects of the transcriptional cascade of events that contribute to ectoderm development and pathogenesis associated with p63 mutations.

Key words: Dlx3, p63, Transcription, Ectodermal dysplasias, Mouse development

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