Mouse keratin 4 is necessary for internal epithelial integrity.

Published

Journal Article

Keratins are intermediate filaments of epithelial cells. Mutations in keratin genes expressed in skin lead to human disorders, including epidermolysis bullosa simplex and epidermolytic hyperkeratosis. We examined the role of keratin 4 (K4) in maintaining the integrity of internal epithelial linings by using gene targeting to generate mice containing a null mutation in the epithelial K4 gene. Homozygous mice that do not express K4 develop a spectrum of phenotypes that affect several organs which express K4 including the esophagus, tongue, and cornea. The cellular phenotypes include basal hyperplasia, lack of maturation, hyperkeratosis, atypical nuclei, perinuclear clearing, and cell degeneration. These results are consistent with the notion that K4 is required for internal epithelial cell integrity. As mutations in K4 in humans lead to a disorder called white sponge nevus, the K4-deficient mice may serve as models for white sponge nevus and for understanding the role of K4 in cellular proliferation and differentiation.

Full Text

Duke Authors

Cited Authors

  • Ness, SL; Edelmann, W; Jenkins, TD; Liedtke, W; Rustgi, AK; Kucherlapati, R

Published Date

  • September 11, 1998

Published In

Volume / Issue

  • 273 / 37

Start / End Page

  • 23904 - 23911

PubMed ID

  • 9727004

Pubmed Central ID

  • 9727004

International Standard Serial Number (ISSN)

  • 0021-9258

Digital Object Identifier (DOI)

  • 10.1074/jbc.273.37.23904

Language

  • eng

Conference Location

  • United States