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Depending on location and activities, <jats:styled-content style=\"fixed-case\">TG<\/jats:styled-content>s are able to modify the signalling, the function and the fate of cells and extracellular connective tissues. Besides mouse models, human diseases enable us to appreciate the function of various <jats:styled-content style=\"fixed-case\">TG<\/jats:styled-content>s. In this study, skin diseases induced by genetic damages or autoimmune targeting of these enzymes will be discussed. <jats:styled-content style=\"fixed-case\">TG<\/jats:styled-content>1, <jats:styled-content style=\"fixed-case\">TG<\/jats:styled-content>3 and <jats:styled-content style=\"fixed-case\">TG<\/jats:styled-content>5 contribute to the cutaneous barrier and thus to the integrity and function of epidermis. <jats:italic><jats:styled-content style=\"fixed-case\">TGM<\/jats:styled-content>1<\/jats:italic> mutations related to autosomal recessive ichthyosis subtypes, <jats:italic><jats:styled-content style=\"fixed-case\">TGM<\/jats:styled-content>5<\/jats:italic> mutations to a mild epidermolysis bullosa phenotype and as novelty <jats:italic><jats:styled-content style=\"fixed-case\">TGM<\/jats:styled-content>3<\/jats:italic> mutation to uncombable hair syndrome will be discussed. Autoimmunity to <jats:styled-content style=\"fixed-case\">TG<\/jats:styled-content>2, <jats:styled-content style=\"fixed-case\">TG<\/jats:styled-content>3 and <jats:styled-content style=\"fixed-case\">TG<\/jats:styled-content>6 may develop in a few of those genetically determined individuals who lost tolerance to gluten, and manifest as coeliac disease, dermatitis herpetiformis or gluten\u2010dependent neurological symptoms, respectively. These gluten responder diseases commonly occur in combination. In autoimmune diseases, the epitope spreading is remarkable, while in some inherited pathologies, a unique compensation of the lost enzyme function is noted.<\/jats:p>","DOI":"10.1111\/exd.13449","type":"journal-article","created":{"date-parts":[[2017,9,23]],"date-time":"2017-09-23T08:02:57Z","timestamp":1506153777000},"page":"807-814","source":"Crossref","is-referenced-by-count":58,"title":["Transglutaminases in autoimmune and inherited skin diseases: The phenomena of epitope spreading and functional compensation"],"prefix":"10.1111","volume":"27","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8472-0712","authenticated-orcid":false,"given":"Sarolta","family":"K\u00e1rp\u00e1ti","sequence":"first","affiliation":[{"name":"Dermatology, Venereology and Dermatooncology Semmelweis University  Budapest Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mikl\u00f3s","family":"S\u00e1rdy","sequence":"additional","affiliation":[{"name":"Dermatology, Venereology and Dermatooncology Semmelweis University  Budapest Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kriszti\u00e1n","family":"N\u00e9meth","sequence":"additional","affiliation":[{"name":"Dermatology, Venereology and Dermatooncology Semmelweis University  Budapest Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bal\u00e1zs","family":"Mayer","sequence":"additional","affiliation":[{"name":"Dermatology, Venereology and Dermatooncology Semmelweis University  Budapest Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Neil","family":"Smyth","sequence":"additional","affiliation":[{"name":"Biological Sciences University of Southampton  Southampton UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mats","family":"Paulsson","sequence":"additional","affiliation":[{"name":"Center for Biochemistry Medical Faculty University of Cologne  Cologne Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heiko","family":"Traupe","sequence":"additional","affiliation":[{"name":"Department of Dermatology University of M\u00fcnster  M\u00fcnster Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2018,2,26]]},"reference":[{"key":"e_1_2_13_2_1","doi-asserted-by":"crossref","first-page":"402","DOI":"10.1055\/s-0038-1642451","volume":"71","author":"Aeschlimann D.","year":"1994","journal-title":"Thromb. 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