Article

Adult mice lacking the p53/p63 target gene PERP are not predisposed to spontaneous tumorigenesis but display features of ectodermal dysplasia syndromes

Stanford University, Stanford, California, United States
Cell Death and Differentiation (Impact Factor: 8.18). 10/2006; 13(9):1614-8. DOI: 10.1038/sj.cdd.4401871
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Available from: Rebecca Ihrie, Apr 11, 2014
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    • "The first cell–cell adhesion gene identified as a p63 direct target was Perp, which encodes a tetraspan membrane protein that localizes to the desmosomes (47). Perp null mice exhibited frequent basal-suprabasal blistering in the skin and oral cavity (47), and developed ectodermal dysplasia and palmoplantar keratoderma (67). A normal pattern of PERP membrane staining was observed in most AEC patients, although in two unrelated patients PERP expression was reduced in the basal and suprabasal layers of the skin (68), consistent with either reduced PERP expression or with protein delocalization due to alterations of other desmosomal components. "
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