Perp is a p63-regulated gene essential for epithelial integrity

Ihrie, R. A., Marques, M. R., Nguyen, B. T., Horner, J. S., Papazoglu, C., Bronson, R. T., Mills, A. A., Attardi, L. D. (March 2005) Perp is a p63-regulated gene essential for epithelial integrity. Cell, 120 (6). pp. 843-856. ISSN 0092-8674

URL: https://www.ncbi.nlm.nih.gov/pubmed/15797384
DOI: 10.1016/j.cell.2005.01.008

Abstract

p63 is a master regulator of stratified epithelial development that is both necessary and sufficient for specitying this multifaceted program. We show here that Perp, a tetraspan membrane protein originally identified as an apoptosis-associated target of the p53 tumor suppressor, is the first direct target of p63 clearly involved in mediating this developmental program in vivo. During embryogenesis, Perp is expressed in an epithelial pattern, and its expression depends on p63. Perp(-/-) mice die postnatally, with dramatic blistering in stratified epithelia symptomatic of compromised adhesion. Perp localizes specifically to desmosomes, adhesion junctions important for tissue integrity, and numerous structural defects in desmosomes are observed in Perp-deficient skin, suggesting a role for Perp in promoting the stable assembly of desmosomal adhesive complexes. These findings demonstrate that Perp is a key effector in the p63 developmental program, playing an essential role in an adhesion subprogram central to epithelial integrity and homeostasis.

Item Type: Paper
Uncontrolled Keywords: p53 dependent apoptosis epidermal morphogenesid P53 homolog P63 cell plakophilin-1 target desmoplakin desmosomes Complex
Subjects: organs, tissues, organelles, cell types and functions > tissues types and functions > embryogenesis
organs, tissues, organelles, cell types and functions > cell types and functions > cell types > epithelial cell
organs, tissues, organelles, cell types and functions > cell types and functions > cell types > epithelial cell
organs, tissues, organelles, cell types and functions > cell types and functions > cell types > epithelial cell

organism description > animal > mammal > rodent > mouse
bioinformatics > genomics and proteomics > genetics & nucleic acid processing > DNA, RNA structure, function, modification > genes, structure and function > genes: types > p63
CSHL Authors:
Communities: CSHL labs > Mills lab
Depositing User: CSHL Librarian
Date: March 2005
Date Deposited: 12 Jan 2012 20:51
Last Modified: 02 Feb 2017 19:59
Related URLs:
URI: http://repository.cshl.edu/id/eprint/22604

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