title: The requirement for perp in postnatal viability and epithelial integrity reflects an intrinsic role in stratified epithelia.
abstract: Mice lacking the desmosome protein Perp exhibit blistering in their stratified epithelia and display postnatal lethality. However, it is unclear if these phenotypes are strictly related to Perp function in stratified epithelia, as Perp expression is not restricted to these tissues during embryogenesis, and certain desmosomal blistering diseases such as pemphigus vulgaris and pemphigus foliaceus have non-cell-intrinsic bases. Furthermore, we show here that Perp is expressed in the heart, raising the possibility that defects in heart function could account for lethality in the Perp-deficient mice. To determine conclusively if Perp function in stratified epithelia is crucial for postnatal survival and epithelial adhesion, we specifically ablated Perp in stratified epithelia by breeding conditional Perp knockout mice to keratin 5 (K5)-Cre transgenic mice. We found that the majority of mice lacking Perp in stratified epithelia die within 10 days after birth, accompanied by blistering and hyperproliferation in the epithelia, similar to the constitutive Perp null mice. Together, these findings indicate that Perp's requirement for both viability and epithelial integrity reflects a role in the stratified epithelial compartment.
authors.affiliation: Department of Radiation Oncology, Division of Radiation and Cancer Biology, Stanford University School of Medicine, Stanford, California 94305, USA.
authors.forename: Michelle R
authors.initials: MR
authors.lastname: Marques
authors.forename: Rebecca A
authors.initials: RA
authors.lastname: Ihrie
authors.forename: Jennifer S
authors.initials: JS
authors.lastname: Horner
authors.forename: Laura D
authors.initials: LD
authors.lastname: Attardi
mesh_terms: D000818:Animals; D002448:Cell Adhesion; D049109:Cell Proliferation; D000078404:Epidermal Cells; D004817:Epidermis / Q000254:growth & development* / Q000378:metabolism*; D005804:Genes, Lethal; D006321:Heart / Q000254:growth & development; D053569:Keratin-15; D053555:Keratin-5; D007633:Keratins / Q000235:genetics; D008565:Membrane Proteins / Q000032:analysis / Q000235:genetics / Q000502:physiology*; D051379:Mice; D018345:Mice, Knockout; D009206:Myocardium / Q000737:chemistry / Q000166:cytology / Q000378:metabolism*
chemical_list: D053569:Keratin-15; D053555:Keratin-5; C492053:Krt15 protein, mouse; D008565:Membrane Proteins; C406964:Perp protein, mouse; D007633:Keratins