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

Mice deficient in involucrin, envoplakin, and periplakin have a defective epidermal barrier. Research Abstract Details 

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Mice deficient in involucrin, envoplakin, and periplakin have a defective epidermal barrier. Abstract Text:

Lisa M Sevilla,Rachida Nachat,Karen R Groot,John F Klement,Jouni Uitto,Philippe Djian,Arto ,Fiona M Watt,

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 gammadelta+ 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.

Mice deficient in involucrin, envoplakin, and periplakin have a defective epidermal barrier. Publishing Authors By Initials

LM Sevilla,R Nachat,KR Groot,JF Klement,J Uitto,P Djian,A ,FM Watt,

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Mice deficient in involucrin, envoplakin, and periplakin have a defective epidermal barrier. Journal Published:

PUBLICATION TYPE: Research Support, Non-U.S. Gov

Journal: The Journal of cell biology

VOLUME: 179

Page Numbers: 1599-612

Journal Abbreviation: J. Cell Biol.

ISSN: 1540-8140

DAY: 31

MONTH: Dec

YEAR: 2007

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

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LANGUAGE: eng

NlmUniqueID: 375356

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Grant and Affiliation Information for Mice deficient in involucrin, envoplakin, and periplakin have a defective epidermal barrier.

AFFILIATION: Cancer Research UK, Cambridge Research Institute, Li Ka Shing Centre, Cambridge CB2 0RE, England, UK.

Country: United States

AGENCY: United States NICHD

GRANT: HD42379-02

ACRONYM: HD

MEDLINETA: J Cell Biol

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