Urwyler-Rosselet, Corinne, Tanghe, Giel ORCID: 0000-0001-5879-7428, Leurs, Kirsten, Gilbert, Barbara, De Rycke, Riet, De Bruyne, Michiel, Lippens, Saskia, Bartunkova, Sonia, De Groote, Philippe, Niessen, Carien, Haftek, Marek, Vandenabeele, Peter ORCID: 0000-0002-6669-8822 and Declercq, Wim (2018). Keratinocyte-Specific Ablation of RIPK4 Allows Epidermal Cornification but Impairs Skin Barrier Formation. J. Invest. Dermatol., 138 (6). S. 1268 - 1279. NEW YORK: ELSEVIER SCIENCE INC. ISSN 1523-1747

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Abstract

In humans, receptor-interacting protein kinase 4 (RIPK4) mutations can lead to the autosomal recessive Bartsocas-Papas and popliteal pterygium syndromes, which are characterized by severe skin defects, pterygia, as well as clefting. We show here that the epithelial fusions observed in RIPK4 full knockout (KO) mice are E-cadherin dependent, as keratinocyte-specific deletion of E-cadherin in RIPK4 full KO mice rescued the tail-to-body fusion and fusion of oral epithelia. To elucidate RIPK4 function in epidermal differentiation and development, we generated epidermis-specific RIPK4 KO mice (RIPK4 EKO). In contrast to RIPK4 full KO epidermis, RIPK4 EKO epidermis was normally stratified and the outside-in skin barrier in RIPK4 EKO mice was largely intact at the trunk, in contrast to the skin covering the head and the outer end of the extremities. However, RIPK4 EKO mice die shortly after birth due to excessive water loss because of loss of tight junction protein claudin-1 localization at the cell membrane, which results in tight junction leakiness. In contrast, mice with keratinocyte-specific RIPK4 deletion during adult life remain viable. Furthermore, our data indicate that epidermis-specific deletion of RIPK4 results in delayed keratinization and stratum corneum maturation and altered lipid organization and is thus indispensable during embryonic development for the formation of a functional inside-out epidermal barrier.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Urwyler-Rosselet, CorinneUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Tanghe, GielUNSPECIFIEDorcid.org/0000-0001-5879-7428UNSPECIFIED
Leurs, KirstenUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gilbert, BarbaraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
De Rycke, RietUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
De Bruyne, MichielUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Lippens, SaskiaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Bartunkova, SoniaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
De Groote, PhilippeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Niessen, CarienUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Haftek, MarekUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Vandenabeele, PeterUNSPECIFIEDorcid.org/0000-0002-6669-8822UNSPECIFIED
Declercq, WimUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-185563
DOI: 10.1016/j.jid.2017.12.031
Journal or Publication Title: J. Invest. Dermatol.
Volume: 138
Number: 6
Page Range: S. 1268 - 1279
Date: 2018
Publisher: ELSEVIER SCIENCE INC
Place of Publication: NEW YORK
ISSN: 1523-1747
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
BARTSOCAS-PAPAS SYNDROME; KAPPA-B ACTIVATION; STRATUM-CORNEUM; TIGHT JUNCTIONS; PROTEIN-KINASE; CORNIFIED ENVELOPE; MICE; DIFFERENTIATION; DEFICIENT; GLUCOSYLCERAMIDESMultiple languages
DermatologyMultiple languages
Refereed: Yes
URI: http://kups.ub.uni-koeln.de/id/eprint/18556

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