We use cookies to ensure that we give you the best experience on our website. By continuing to browse this repository, you give consent for essential cookies to be used. You can read more about our Privacy and Cookie Policy.

Durham Research Online
You are in:

Mice deficient in involucrin, envoplakin and periplakin have a defective epidermal barrier

Sevilla, L.M. and Nachat, R. and Groot, K.R. and Klement, J.F. and Uitto, J. and Djian, P. and Määttä, A. and Watt, F.M. (2007) 'Mice deficient in involucrin, envoplakin and periplakin have a defective epidermal barrier.', Journal of cell biology., 179 (7). pp. 1599-1612.


The cornified envelope is assembled from transglutaminase cross-linked proteins and lipids in the outermost epidermal layers and is essential for skin barrier function. Involucrin, envoplakin, and periplakin form the protein scaffold on which the envelope assembles. To examine their combined function, we generated mice deficient in all three genes. The triple knockouts have delayed embryonic barrier formation and postnatal hyperkeratosis (abnormal accumulation of cornified cells) resulting from impaired desquamation. Cornified envelopes form but are ultrastructurally abnormal, with reduced lipid content and decreased mechanical integrity. Expression of proteases is reduced and the protease inhibitor, serpina1b, is highly upregulated, resulting in defective filaggrin processing and delayed degradation of desmoglein 1 and corneodesmosin. There is infiltration of CD4+ T cells and a reduction in resident γδ+ T cells, reminiscent of atopic dermatitis. Thus, combined loss of the cornified envelope proteins not only impairs the epidermal barrier, but also changes the composition of T cell subpopulations in the skin.

Item Type:Article
Additional Information:Supplemental Material can be found at:
Full text:(VoR) Version of Record
Download PDF
Publisher Web site:
Publisher statement:This article is published under the Creative Commons Attribution (CC-BY) license.
Date accepted:No date available
Date deposited:12 June 2012
Date of first online publication:December 2007
Date first made open access:No date available

Save or Share this output

Look up in GoogleScholar