title: Gene regulatory mechanisms orchestrated by p63 in epithelial development and related disorders.
abstract: The transcription factor p63 belongs to the p53 family and is a key regulator in epithelial commitment and development. Mutations in p63 give rise to several epithelial related disorders with defects in skin, limb and orofacial structures. Since the discovery of p63, efforts have been made to identify its target genes using individual gene approaches and to understand p63 function in normal epithelial development and related diseases. Recent genome-wide approaches have identified tens of thousands of potential p63-regulated target genes and regulatory elements, and reshaped the concept of gene regulation orchestrated by p63. These data also provide insights into p63-related disease mechanisms. In this review, we discuss the regulatory role of p63 in normal and diseased epithelial development in light of these novel findings. We also propose future perspectives for dissecting the molecular mechanism of p63-mediated epithelial development and related disorders as well as for potential therapeutic strategies.
authors: Radboud University, Department of Molecular Developmental Biology, Faculty of Science, Radboud Institute for Molecular Life Sciences, Nijmegen, The Netherlands. Electronic address: e.kouwenhoven@science.ru.nl.
authors: Evelyn N
authors: EN
authors: Kouwenhoven
authors: Radboud university medical center, Department of Human Genetics, Radboud Institute for Molecular Life Sciences, Nijmegen, The Netherlands. Electronic address: hans.vanbokhoven@radboudumc.nl.
authors: Hans
authors: H
authors: van Bokhoven
authors: Radboud University, Department of Molecular Developmental Biology, Faculty of Science, Radboud Institute for Molecular Life Sciences, Nijmegen, The Netherlands; Radboud university medical center, Department of Human Genetics, Radboud Institute for Molecular Life Sciences, Nijmegen, The Netherlands. Electronic address: jo.zhou@radboudumc.nl.
authors: Huiqing
authors: H
authors: Zhou
mesh_terms: D004268:DNA-Binding Proteins / Q000235:genetics*; D004848:Epithelium / Q000254:growth & development / Q000378:metabolism*; D018507:Gene Expression Regulation, Developmental; D053263:Gene Regulatory Networks; D018390:Gene Targeting; D030342:Genetic Diseases, Inborn / Q000235:genetics / Q000473:pathology; D006801:Humans; D008565:Membrane Proteins / Q000096:biosynthesis / Q000235:genetics* / Q000378:metabolism
chemical_list: C081876:CKAP4 protein, human; D004268:DNA-Binding Proteins; D008565:Membrane Proteins
keywords: Epithelial development; Epithelial-related disease; Gene regulation; p63