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, Anne Puel15-17, Christine Bodemer1,2* and Asma Smahi2* of Dermatology, Reference Center for Genodermatoses (MAGEC), NeckerEnfants Malades Hospital (AP-HP), vol.12, pp.15-18

. M3c-necker, Pediatric Reference Center for Inherited Cardiac Diseases, Necker-Enfants Malades Hospital (AP-HP)

, Molecular & Cellular Physiology and Photon Science

, Laboratory of Human Genetics of Infectious Diseases

A. Smahi, I. Inserm-u1163, and . Institute, 24 boulevard Montparnasse, 75015 Paris. asma.smahi@inserm.fr; tel.: +33 142 754 278; fax: +33 142 754 217 and Christine Bodemer, Department of Dermatology and Referral Center for Genodermatoses (MAGEC), Imagine Institute, Necker-Enfants Malades Hospital, APHP and Paris Descartes-Sorbonne Cité University, 149 rue de Sèvres, F-75743 Paris cedex15, France. christine.bodemer@nck.aphp.fr

, 3311 Figures: 3 Supplementary Figures, p.7

, Funding sources: grants from the French Society of Dermatology (SFD), from Laboratoires Expanscience and from French National Research Agency (ANR) under the "Investments for the future" program (grant

, Conflict of Interest: the authors have declared that no conflict of interest exists

G. Courtois and F. ). , 16 hours after transfection, cells were stimulated with 10 ng/ml IL-1? or 20 ng/ml TNF?. 8 hours after stimulation, luciferase activity was determined using a dual luciferase assay kit

, Addgene) or with 250 ng of DSP plasmid (plasmid #32227, Addgene), together with 0.2?g of Ig?luc (see above in the "Luciferase NF-?B reporter assays" section), and then lysed in RIPA buffer, Immunoblotting analysis HEK293T cells were transiently transfected with increasing doses (100-1000ng) of DSG1 plasmid (plasmid #55029

, Bound antibodies were visualized with horseradish-peroxidase-conjugated antibodies against rabbit or mouse IgG (Santa Cruz Biotechnology) and an Enhanced Chemiluminescence kit (SuperSignal West Dura Extended Duration Substrate, Western blotting was performed using mouse anti-DSP I/II antibody (diluted 1:1000, sc-390975

, For virus preparation, pRetro-DSG1 or blank vector were co-transfected using jetPRIMETM reagent (Polyplus Transfection Inc.) and packaging vectors pGag/Pol and pVSVG into HEK293T cells, Retroviral vector production pRetro-DSG1 was sent as a gift by Kathleen Green

, 12 hours later, keratinocytes (20% confluent) were infected with retrovirus containing (or not) the DSG1 construct. 24 hours after infection, keratinocytes were stimulated with 10 ng/ml of IL-223

, 24 hours after stimulation, the cells were pelleted for RNA extraction, p.1

, The secondary antibodies were antirabbit Alexa Fluor 546 and anti-mouse Alexa Fluor 488 (Life Technologies, Immunohistochemical reactions were performed on 4-?m-thick frozen tissue sections using rabbit anti-DSG1 antibody

, Images were acquired and processed with an LSM700 microscope (Zeiss, Coverslips were mounted with mounting medium with DAPI (Duolink, Olink Biosciences

, Light microscopy Skin and heart biopsy specimens were fixed in 10% formalin, embedded in paraffin and processed using standard procedures. Three-?m-thick sections were stained with H&E reagent and examined under the LEICA DFC280 light microscopy

T. Tecnai, . Fei, and O. Hillsboro, Ultrathin sections were prepared and stained with uranyl acetate and lead citrate for electron microscopy

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