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Structural organization of cornified cell envelopes and alterations in inherited skin disorders
Author(s) -
IshidaYamamoto Akemi,
Iizuka Hajime
Publication year - 1998
Publication title -
experimental dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.108
H-Index - 96
eISSN - 1600-0625
pISSN - 0906-6705
DOI - 10.1111/j.1600-0625.1998.tb00295.x
Subject(s) - loricrin , involucrin , microbiology and biotechnology , biology , filaggrin , gene , genetics , cellular differentiation , immunology , atopic dermatitis
The cornified cell envelope is a highly insoluble and extremely tough structure formed beneath the cell membrane during terminal differentiation of kerationcytes. Its main function is to provide human skin with a protective barrier against the environment. Sequential cross‐linking of several integral components catalyzed by transglutaminases leads to a gradual increase in the thickness of the envelope and underscores its rigidity. Key structural players in this cross‐linking process include involucrin, loricrin, SPRRs, elafin, cystatin A, S100 family proteins, and some desmosomal proteins. The recent identification of genetic skin diseases with mutations in the genes encoding some of these proteins, including transglutaminase 1 and loricrin, has disclosed that abnormal cornified cell envelope synthesis is significantly involved in the pathophysiology of certain inherited keratodermas and reflects perturbations in the complex yet highly orderly process of cornified cell envelope formation in normal skin biology.