Gene expression data from the skin of Cdsniep-/-adult mice

Peeling Skin Disease (PSD; OMIM 270300) is an inflammatory ichthyosis due to homozygous loss-of-function mutations in Corneodesmosin (CDSN) and characterized by lifelong patchy peeling of the skin associated with eczema, food allergy and severe itching. The pathophysiology of PSD is still poorly known. The initial event of the disease, the detachment of the SC due to a CDSN deficiency, leads to an impairment of the permeability barrier which could in turn trigger erythema, pruritus and atopic manifestations by mechanisms not entirely elucidated. Cdsn-deficient mouse models are interesting tools to decipher these underlying mechanisms. In order to explore the still poorly known pathophysiological mechanisms of PSD, we analyzed the cutaneous transcriptome of two epidermis-specific Cdsn-deficient mouse models representative of early (occurrence of the permeability defect in Cdsnep-/- E18.5 embryos) and chronic (permanent permeability defect in Cdsniep-/- adult mice) phases of the disease We used affymetrix microarrays to explore genes expression in the skin of Cdsniep-/- adult mice which represent the chronic phase of the disease. Cdsniep-/- mice are epidermis-specific Cdsn-deficient and their genotype is KRT14-CreERT2(tg/0)/Cdsnfl/fl. To induce Cdsn inactivation and generate Cdsniep-/- mice (KO), seven to 12-week-old KRT14-CreERT2(tg/0)/Cdsnfl/fl mice were treated by daily topical application of 4-hydroxytamoxifen (4-OHTam, 100μg/ear/day) on both ears until fifteen continuous days. KRT14-CreERT2(tg/0)/Cdsnwt/wt treated in the same conditions were used as control (WT). Total RNA were then extracted from the skin of ears. Microarray experiments were performed with RNAs from 4 individuals KO and from 4 individuals WT.