Lessons from loricrin-deficient mice: compensatory mechanisms maintaining skin barrier function in the absence of a major cornified envelope protein

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Etat: Public
Version: de l'auteur⸱e
ID Serval
serval:BIB_377ED538A3F4
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Institution
Titre
Lessons from loricrin-deficient mice: compensatory mechanisms maintaining skin barrier function in the absence of a major cornified envelope protein
Périodique
Journal of Cell Biology
Auteur⸱e⸱s
Koch  P. J., de Viragh  P. A., Scharer  E., Bundman  D., Longley  M. A., Bickenbach  J., Kawachi  Y., Suga  Y., Zhou  Z., Huber  M., Hohl  D., Kartasova  T., Jarnik  M., Steven  A. C., Roop  D. R.
ISSN
0021-9525 (Print)
Statut éditorial
Publié
Date de publication
10/2000
Volume
151
Numéro
2
Pages
389-400
Notes
Journal Article
Research Support, Non-U.S. Gov't
Research Support, U.S. Gov't, P.H.S. --- Old month value: Oct 16
Résumé
The epidermal cornified cell envelope (CE) is a complex protein-lipid composite that replaces the plasma membrane of terminally differentiated keratinocytes. This lamellar structure is essential for the barrier function of the skin and has the ability to prevent the loss of water and ions and to protect from environmental hazards. The major protein of the epidermal CE is loricrin, contributing approximately 70% by mass. We have generated mice that are deficient for this protein. These mice showed a delay in the formation of the skin barrier in embryonic development. At birth, homozygous mutant mice weighed less than control littermates and showed skin abnormalities, such as congenital erythroderma with a shiny, translucent skin. Tape stripping experiments suggested that the stratum corneum stability was reduced in newborn Lor(-/-) mice compared with wild-type controls. Isolated mutant CEs were more easily fragmented by sonication in vitro, indicating a greater susceptibility to mechanical stress. Nevertheless, we did not detect impaired epidermal barrier function in these mice. Surprisingly, the skin phenotype disappeared 4-5 d after birth. At least one of the compensatory mechanisms preventing a more severe skin phenotype in newborn Lor(-/-) mice is an increase in the expression of other CE components, such as SPRRP2D and SPRRP2H, members of the family of "small proline rich proteins", and repetin, a member of the "fused gene" subgroup of the S100 gene family.
Mots-clé
Adaptation, Biological Amino Acid Sequence Animals Biomechanics Cell Membrane Cloning, Molecular Epidermis/*physiology Intermediate Filament Proteins/biosynthesis Membrane Proteins/deficiency/*genetics Mice Mice, Inbred BALB C Mice, Inbred C57BL Mice, Knockout Molecular Sequence Data Permeability S100 Proteins/biosynthesis Skin Physiology/*genetics Up-Regulation
Pubmed
Web of science
Open Access
Oui
Création de la notice
25/01/2008 16:36
Dernière modification de la notice
20/08/2019 13:25
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