Altered epidermal lipid processing and calcium distribution in the KID syndrome mouse model Cx26S17F

•Disturbed epidermal lipid secretion and location in Cx26S17F KID syndrome mouse model.•Loss of ω-esterified ceramides from the epidermal surface of Cx26S17F mice.•Cx26S17F leads to an altered epidermal calcium gradient. The keratitis–ichthyosis–deafness (KID) syndrome is caused by mutations in the...

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Veröffentlicht in:	FEBS letters 2015-07, Vol.589 (15), p.1904-1910
Hauptverfasser:	Bosen, Felicitas, Celli, Anna, Crumrine, Debra, vom Dorp, Katharina, Ebel, Philipp, Jastrow, Holger, Dörmann, Peter, Winterhager, Elke, Mauro, Theodora, Willecke, Klaus
Format:	Artikel
Sprache:	eng
Schlagworte:	animal models Animals calcium Calcium - metabolism ceramides Connexin 26 Connexins - genetics Deafness - metabolism Disease Models, Animal Epidermal calcium gradient Epidermal ceramides Epidermal water barrier defect Epidermis - metabolism Female hearing heterozygosity humans hyperplasia Ichthyosis - metabolism Keratitis - metabolism Keratitis–ichthyosis–deafness syndrome Lipid Metabolism Male Mice Microscopy, Fluorescence mutants mutation neonates secretion Transgenic mouse mutant
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