Abnormal keratin 1 and 10 cytoskeleton in cultured keratinocytes from epidermolytic hyperkeratosis caused by keratin 10 mutations.

Détails

ID Serval
serval:BIB_A864A8A184D1
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Institution
Titre
Abnormal keratin 1 and 10 cytoskeleton in cultured keratinocytes from epidermolytic hyperkeratosis caused by keratin 10 mutations.
Périodique
Journal of Investigative Dermatology
Auteur(s)
Huber M., Scaletta C., Benathan M., Frenk E., Greenhalgh D.A., Rothnagel J.A., Roop D.R., Hohl D.
ISSN
0022-202X
Statut éditorial
Publié
Date de publication
05/1994
Peer-reviewed
Oui
Volume
102
Numéro
5
Pages
691-694
Langue
anglais
Notes
Publication types: Journal Article ; Research Support, Non-U.S. Gov't ; Research Support, U.S. Gov't, P.H.S. - Publication Status: ppublish
Résumé
Epidermolytic hyperkeratosis is caused by mutations of the differentiation-specific keratins K1 and K10. These mutations produce a weakened cytoskeleton that is prone to collapse resulting in cell fragility and lysis. In this study we have analyzed cultured keratinocytes from EHK patients bearing 10R-to-H and 15L-to-S mutations within the 1A segment of the K10 rod domain. Keratinocytes were grown submerged in serum-free medium and induced to differentiate by growing to confluence and increasing the Ca++ concentration in the medium. Cultures were either harvested for mRNA sequence analysis or subjected to immunofluorescence microscopy. Differentiating keratinocytes from these patients were found to express these K10 mutations in their mRNA. Moreover, these cells could be distinguished from normal keratinocytes by their aberrant morphology. EHK keratinocytes frequently exhibited a collapsed perinuclear network of K1/K10 filaments and sometimes peripheral granules of K1 and K10 aggregates, reminiscent of the cells of the suprabasal layers in these patients. This report documents the expression of mutant keratin 10 in cultured EHK keratinocytes.
Mots-clé
Base Sequence, Cell Division, Cells, Cultured, Cytoskeleton, Humans, Hyperkeratosis, Epidermolytic, Keratin-10, Keratinocytes, Keratins, Molecular Sequence Data, Mutation, RNA, Messenger
Pubmed
Web of science
Open Access
Oui
Création de la notice
25/01/2008 17:36
Dernière modification de la notice
20/08/2019 16:12
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