RESEARCH ARTICLE


Corneodesmosin: Structure, Function and Involvement in Pathophysiology



Nathalie Jonca, Cecile Caubet, Marina Guerrin, Michel Simon, Guy Serre*
CNRS – University Toulouse III, UMR 5165 “Epidermis Differentiation and Rheumatoid Autoimmunity”, IFR 150 (INSERM-CNRS-University Toulouse III-CHU), France


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Creative Commons License
© 2010 Jonca et al.

open-access license: This is an open access article distributed under the terms of the Creative Commons Attribution 4.0 International Public License (CC-BY 4.0), a copy of which is available at: https://creativecommons.org/licenses/by/4.0/legalcode. This license permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

* Address correspondence to this author at the CNRS-UPS UMR5165, CHU Purpan, Place du Docteur Baylac, TSA 40031, 31059 Toulouse Cedex 9, France; Tel: 33 5 6115 8402; Fax: 33 5 6149 9036; E-mail: guy.serre@udear.cnrs.fr


Abstract

Corneodesmosin (CDSN) was identified in the early 90th by raising monoclonal antibodies against human plantar stratum corneum. It is a protein specific to desmosomes that will undergo transformation into corneodesmosomes, i.e. in man, desmosomes of the epidermis, of the three epithelial layers of the inner root sheath of the hair follicles and of the hard palate epithelium. After its secretion by granular keratinocytes via the lamellar bodies, CDSN is incorporated into the desmoglea of the desmosomes shortly before their transformation into corneodesmosomes. CDSN displays adhesive properties, mostly attributable to its N-terminal glycine-rich domain, and is sequentially proteolyzed as corneocytes migrate towards the skin surface. The recent inactivation of Cdsn in mice induced a lethal epidermal barrier disruption and hair follicle degeneration related to desmosome dysfunction, confirming the essential role of the protein in epidermis and hair follicle integrity. CDSNCDSN is located on chromosome 6, in the major psoriasis susceptibility locus PSORS1. Intriguingly, the only monogenic disease identified so far associated with nonsense mutations in CDSN, leading to the formation of a truncated protein, is a rare autosomal dominant disease, hypotrichosis simplex of the scalp. In this review, we expose data from the discovery of the protein to the most recent findings related to the relationship between its structure and function. In particular, the important benefits of mouse models and human diseases for the comprehension of CDSN role in the epidermis and hair follicles are reported in details.

Keywords: Corneodesmosome, corneodesmosin, cornification, desquamation, differentiation, epidermis, hyperkeratosis, hypotrichosis, kallikrein, psoriasis, stratum corneum.