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Titolo:
Normal ultrastructure, but altered stratum corneum lipid and protein composition in a mouse model for epidermolytic hyperkeratosis
Autore:
Reichelt, J; Doering, T; Schnetz, E; Fartasch, M; Sandhoff, K; Magin, TM;
Indirizzi:
Univ Bonn, Inst Genet, Abt Mol Genet, D-53117 Bonn, Germany Univ Bonn Bonn Germany D-53117 net, Abt Mol Genet, D-53117 Bonn, Germany Univ Bonn, Bonner Forum Biomed, D-53117 Bonn, Germany Univ Bonn Bonn Germany D-53117 onner Forum Biomed, D-53117 Bonn, Germany Univ Bonn, Kekule Inst Organ Chem & Biochem, D-5300 Bonn, Germany Univ Bonn Bonn Germany D-5300 Organ Chem & Biochem, D-5300 Bonn, Germany Univ Erlangen Nurnberg, Dermatol Klin, D-8520 Erlangen, Germany Univ Erlangen Nurnberg Erlangen Germany D-8520 D-8520 Erlangen, Germany
Titolo Testata:
JOURNAL OF INVESTIGATIVE DERMATOLOGY
fascicolo: 3, volume: 113, anno: 1999,
pagine: 329 - 334
SICI:
0022-202X(199909)113:3<329:NUBASC>2.0.ZU;2-N
Fonte:
ISI
Lingua:
ENG
Soggetto:
CORNIFIED CELL-ENVELOPE; KERATIN INTERMEDIATE FILAMENTS; PERMEABILITY BARRIER; KNOCKOUT MICE; GENETIC-BASIS; EPIDERMIS; SKIN; EXPRESSION; DIFFERENTIATION; CERAMIDES;
Keywords:
ceramide; involucrin; keratin 10; lamellar body;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
50
Recensione:
Indirizzi per estratti:
Indirizzo: Magin, TM Univ Bonn, Inst Genet, Abt Mol Genet, Romerstr 164, D-53117 Bonn, Germany Univ Bonn Romerstr 164 Bonn Germany D-53117 53117 Bonn, Germany
Citazione:
J. Reichelt et al., "Normal ultrastructure, but altered stratum corneum lipid and protein composition in a mouse model for epidermolytic hyperkeratosis", J INVES DER, 113(3), 1999, pp. 329-334

Abstract

Recently, we established keratin 10-deficient mice, serving as a model forthe hyperkeratotic skin disorder epidermolytic hyperkeratosis. The considerable ichthyosis in these mice suggested alterations in terminal differentiation and in the formation of a functional epidermal barrier. Here, we report on the ultrastructural organization and composition of the stratum corneum lipids and on the expression of two major cornified envelope proteins. Electron microscopy of ruthenium tetroxide postfixed skin samples demonstrated a normal extrusion and morphology of lamellar bodies as well as the formation of bona fide lamellar layers in neonatal keratin 10-deficient mice. When we studied the composition of the major stratum corneum Lipids, however, we found significant changes. Most importantly, the analysis of ceramide subpopulations revealed that the total amount of ceramide 2 was elevated inkeratin 10-deficient mice, whereas ceramides 1, 3, 4, and 5 were decreasedamong total stratum corneum Lipids. The amount of the ceramide precursors sphingomyelin and glucosylceramide was reduced in the stratum corneum without accompanying changes in the mRNA coding for acid sphingomyelinase, Notably, we found an increased mRNA and protein content for involucrin in neonatal keratin 10-deficient mice, whereas the expression of loricrin was not changed. Our data demonstrate that, although the formation of lipid layers inthe stratum corneum appeared to be normal, its lipid composition is significantly altered in keratin 10-deficient mice.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 26/09/20 alle ore 13:23:54