Structural organization of cornified cell envelopes and alterations in inherited skin disorders.

Abstract	The cornified cell envelope is a highly insoluble and extremely tough structure formed beneath the cell membrane during terminal differentiation of keratinocytes. 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.
Authors	A Ishida-Yamamoto, H Iizuka
Journal	Experimental dermatology (Exp Dermatol) Vol. 7 Issue 1 Pg. 1-10 (Feb 1998) ISSN: 0906-6705 [Print] Denmark
PMID	9517915 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Review)
Chemical References	Cornified Envelope Proline-Rich Proteins Cystatins Filaggrin Proteins Intermediate Filament Proteins Membrane Proteins Protein Precursors Proteinase Inhibitory Proteins, Secretory Proteins loricrin involucrin Transglutaminases
Topics	Amino Acid Sequence Animals Cell Membrane (metabolism, pathology) Cornified Envelope Proline-Rich Proteins Cystatins (metabolism) Filaggrin Proteins Humans Ichthyosis (genetics, metabolism, pathology) Intermediate Filament Proteins (metabolism) Keratinocytes (metabolism, pathology) Keratoderma, Palmoplantar, Diffuse (genetics, metabolism, pathology) Membrane Proteins (genetics, metabolism) Protein Precursors (metabolism) Proteinase Inhibitory Proteins, Secretory Proteins (metabolism) Skin (cytology, metabolism) Skin Diseases, Genetic (genetics, metabolism, pathology) Transglutaminases (metabolism)

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