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001     137318
005     20240321220228.0
024 7 _ |a 10.1038/jid.2013.416
|2 doi
024 7 _ |a pmid:24121403
|2 pmid
024 7 _ |a 0022-202X
|2 ISSN
024 7 _ |a 1523-1747
|2 ISSN
037 _ _ |a DZNE-2020-03640
041 _ _ |a English
082 _ _ |a 610
100 1 _ |a Bär, Janina
|0 P:(DE-HGF)0
|b 0
245 _ _ |a Skin fragility and impaired desmosomal adhesion in mice lacking all keratins.
260 _ _ |a Amsterdam
|c 2014
|b Elsevier
264 _ 1 |3 print
|2 Crossref
|b Elsevier BV
|c 2014-04-01
336 7 _ |a article
|2 DRIVER
336 7 _ |a Output Types/Journal article
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336 7 _ |a Journal Article
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|s 1708431338_6810
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336 7 _ |a ARTICLE
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336 7 _ |a JOURNAL_ARTICLE
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336 7 _ |a Journal Article
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520 _ _ |a Keratins perform major structural and regulatory functions in epithelia. Owing to redundancy, their respective contribution to epidermal integrity, adhesion, and cell junction formation has not been addressed in full. Unexpectedly, the constitutive deletion of type II keratins in mice was embryonic lethal ∼ E9.5 without extensive tissue damage. This prompted us to analyze keratin functions in skin where keratins are best characterized. Here, we compare the mosaic and complete deletion of all type II keratins in mouse skin, with distinct consequences on epidermal integrity, adhesion, and organismal survival. Mosaic knockout (KO) mice survived ∼ 12 days while global KO mice died perinatally because of extensive epidermal damage. Coinciding with absence of keratins, epidermal fragility, inflammation, increased epidermal thickness, and increased proliferation were noted in both strains of mice, accompanied by significantly smaller desmosomes. Decreased desmosome size was due to accumulation of desmosomal proteins in the cytoplasm, causing intercellular adhesion defects resulting in intercellular splits. Mixing different ratios of wild-type and KO keratinocytes revealed that ∼ 60% of keratin-expressing cells were sufficient to maintain epithelial sheets under stress. Our data reveal a major contribution of keratins to the maintenance of desmosomal adhesion and epidermal integrity with relevance for the treatment of epidermolysis bullosa simplex and other keratinopathies.
536 _ _ |a 342 - Disease Mechanisms and Model Systems (POF3-342)
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|f POF III
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542 _ _ |i 2014-04-01
|2 Crossref
|u https://www.elsevier.com/tdm/userlicense/1.0/
542 _ _ |i 2015-12-02
|2 Crossref
|u https://www.elsevier.com/open-access/userlicense/1.0/
588 _ _ |a Dataset connected to CrossRef, PubMed,
650 _ 7 |a Keratins, Type II
|2 NLM Chemicals
650 _ 2 |a Animals
|2 MeSH
650 _ 2 |a Cell Adhesion
|2 MeSH
650 _ 2 |a Cell Membrane: metabolism
|2 MeSH
650 _ 2 |a Cytoplasm: metabolism
|2 MeSH
650 _ 2 |a Desmosomes: metabolism
|2 MeSH
650 _ 2 |a Disease Models, Animal
|2 MeSH
650 _ 2 |a Epidermis: metabolism
|2 MeSH
650 _ 2 |a Epidermolysis Bullosa Simplex: metabolism
|2 MeSH
650 _ 2 |a Epithelium: metabolism
|2 MeSH
650 _ 2 |a Gene Deletion
|2 MeSH
650 _ 2 |a Gene Expression Regulation, Developmental
|2 MeSH
650 _ 2 |a Keratinocytes: metabolism
|2 MeSH
650 _ 2 |a Keratins, Type II: genetics
|2 MeSH
650 _ 2 |a Keratins, Type II: metabolism
|2 MeSH
650 _ 2 |a Mice
|2 MeSH
650 _ 2 |a Mice, Knockout
|2 MeSH
650 _ 2 |a Mosaicism
|2 MeSH
650 _ 2 |a Phenotype
|2 MeSH
650 _ 2 |a Skin: embryology
|2 MeSH
650 _ 2 |a Skin: pathology
|2 MeSH
700 1 _ |a Kumar, Vinod
|0 P:(DE-HGF)0
|b 1
700 1 _ |a Roth, Wera
|0 P:(DE-2719)2810635
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700 1 _ |a Schwarz, Nicole
|0 P:(DE-HGF)0
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700 1 _ |a Richter, Miriam
|0 P:(DE-HGF)0
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700 1 _ |a Leube, Rudolf E
|0 P:(DE-HGF)0
|b 5
700 1 _ |a Magin, Thomas M
|0 P:(DE-HGF)0
|b 6
|e Corresponding author
773 1 8 |a 10.1038/jid.2013.416
|b : Elsevier BV, 2014-04-01
|n 4
|p 1012-1022
|3 journal-article
|2 Crossref
|t Journal of Investigative Dermatology
|v 134
|y 2014
|x 0022-202X
773 _ _ |a 10.1038/jid.2013.416
|g Vol. 134, no. 4, p. 1012 - 1022
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|q 134:4<1012 - 1022
|p 1012-1022
|t The journal of investigative dermatology
|v 134
|y 2014
|x 0022-202X
856 4 _ |u https://pub.dzne.de/record/137318/files/DZNE-2020-03640_Restricted.pdf
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910 1 _ |a Deutsches Zentrum für Neurodegenerative Erkrankungen
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