Author:
Keuylian Zela,Hovnanian Alain
Abstract
Abstract
Protease regulation plays a crucial role in skin homeostasis and inflammation as revealed by the identification of loss-of-function mutations in SPINK5 (serine protease inhibitor of Kazal type 5) in Netherton sydrome (NS). SPINK5 encodes LEKTI (lympho-epithelial Kazal type related inhibitor), a multidomain serine protease inhibitor expressed in all stratified epithelia. Our laboratory has developed a number of murine models which have been instrumental in dissecting the pathogenesis of NS. This minireview discusses the major findings of these models and emphasizes the role of protease regulation, especially kallikrein-related peptidases in NS.
Funder
Agence Nationale de la Recherche
Subject
Clinical Biochemistry,Molecular Biology,Biochemistry
Reference62 articles.
1. PAR2 absence completely rescues inflammation and ichthyosis caused by altered CAP1/Prss8 expression in mouse skin;Nat. Commun.,2011
2. Altered lamellar body secretion and stratum corneum membrane structure in Netherton syndrome;Arch. Dermatol.,1999
3. KLK5 inactivation reverses cutaneous hallmarks of Netherton syndrome;PLoS Genet.,2015
4. A potential role for multiple tissue kallikrein serine proteases in epidermal desquamation;J. Biol. Chem.,2007
5. Transgenic kallikrein 5 mice reproduce major cutaneous and systemic hallmarks of Netherton syndrome;J. Exp. Med.,2014
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