TP63 (tumor protein 63) acts as a sequence-specific DNA binding transcriptional activator or repressor. The isoforms contain a varying set of transactivation and auto-regulating transactivation inhibiting domains thus showing an isoform-specific activity. Isoform 2 activates RIPK4 transcription. TP63 may be required in conjunction with TP73/p73 for initiation of p53/TP53 dependent apoptosis in response to genotoxic insults and the presence of activated oncogenes. It is involved in Notch signaling by inducing JAG1 and JAG2. TP63 plays a role in the regulation of epithelial morphogenesis. The ratio of DeltaN-type and TA*-type isoforms may govern the maintenance of epithelial stem cell compartments and regulate the initiation of epithelial stratification from the undifferentiated embryonal ectoderm. TP63 is required for limb formation from the apical ectodermal ridge. It also activates transcription of the p21 promoter. Mutations affecting the gene can result in Acro-dermato-ungual-lacrimal-tooth syndrome, ankyloblepharon-ectodermal defects-cleft lip/palate, ectrodactyly ectodermal dysplasia, split-hand/foot malformation 4, limb-mammary syndrome, ectodermal dysplasia - Rapp-Hodgkin type, and orofacial cleft 8.
amplified in squamous cell carcinoma; Chronic ulcerative stomatitis protein; CUSP; Keratinocyte transcription factor KET; KET; p40; p51; p63; p73L; TP63; Transformation-related protein 63; Tumor protein 63; tumor protein p53-competing protein; Tumor protein p73-like; P63; P73H; P73L; TP73L