{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,11]],"date-time":"2025-12-11T14:18:20Z","timestamp":1765462700505,"version":"3.48.0"},"reference-count":32,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2025,10,23]],"date-time":"2025-10-23T00:00:00Z","timestamp":1761177600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Deutsche Derma Gesell"],"published-print":{"date-parts":[[2025,12]]},"abstract":"<jats:title>Summary<\/jats:title>\n                  <jats:p>\n                    Netherton syndrome (NTS) is a syndromic ichthyosis characterized by scaling, erythroderma, and proneness towards allergies and infections. It is caused by mutations in the\n                    <jats:italic>SPINK5<\/jats:italic>\n                    gene, encoding the lympho\u2010epithelial Kazal\u2010type 5 related inhibitor (LEKTI), which regulates the activity of kallikreins in the skin. Treatment options are limited, insufficient, time\u2010consuming and a financial burden for the patients.\n                  <\/jats:p>\n                  <jats:p>In the last years, genotype\u2010phenotype correlations in NTS and immunological phenotypes have been delineated. Psoriasiform patterns have been described. Moreover, the literature reports on biological therapies as novel therapeutic options in NTS. Previously, recombinant human LEKTI domain\u00a08+9 (rhLEKTI 8+9) was identified as a potent kallikrein inhibitor, implying that these subunits are suitable for a topical protein substitution therapy.<\/jats:p>\n                  <jats:p>We aimed to characterize NTS in more detail to discuss further personalized therapeutic options by phenotyping a large cohort of NTS patients and performing serum analyses. Moreover, we worked on preliminary steps for a protein replacement therapy by investigating recombinant expressed rhLEKTI8+9 and establishing 3D epidermal equivalents for therapeutic evaluation.<\/jats:p>","DOI":"10.1111\/ddg.15928","type":"journal-article","created":{"date-parts":[[2025,10,23]],"date-time":"2025-10-23T07:32:39Z","timestamp":1761204759000},"page":"1528-1532","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Immunological phenotype and skin modeling of Netherton syndrome"],"prefix":"10.1111","volume":"23","author":[{"given":"Kira","family":"S\u00fc\u00dfmuth","sequence":"first","affiliation":[{"name":"Department of Dermatology and Allergology Helios Klinikum Berlin\u2010Buch Campus of Medical School Berlin  Berlin Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Heiko","family":"Traupe","sequence":"additional","affiliation":[{"name":"Department of Dermatology University Hospital M\u00fcnster  M\u00fcnster Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Helmut","family":"Wittkowski","sequence":"additional","affiliation":[{"name":"Department of Pediatric Rheumatology and Immunology University Children's Hospital M\u00fcnster  M\u00fcnster Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Judith","family":"Fischer","sequence":"additional","affiliation":[{"name":"Institute of Human Genetics Faculty of Medicine Medical Center \u2013 University of Freiburg University of Freiburg  Freiburg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vinzenz","family":"Oji","sequence":"additional","affiliation":[{"name":"Department of Dermatology University Hospital M\u00fcnster  M\u00fcnster Germany"},{"name":"Hautarztpraxis am Buddenturm  M\u00fcnster Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jacqueline","family":"Bodes","sequence":"additional","affiliation":[{"name":"Department of Dermatology University Hospital M\u00fcnster  M\u00fcnster Germany"},{"name":"Institute of Pharmaceutical Technology and Biopharmaceutics University of M\u00fcnster  M\u00fcnster Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2025,10,23]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2009.06.009"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1515\/hsz-2014-0137"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1080\/21678707.2020.1857724"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.17203"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.16882"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/ddg.13340"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41572-022-00412-3"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/jdv.19804"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-022-02430-6"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.3389\/fped.2022.1074243"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamadermatol.2020.1019"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1177\/03946320231172881"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00403-022-02325-3"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/1346-8138.17449"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.19546"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2013.08.003"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.3390\/pharmaceutics13122012"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcyt.2020.10.001"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.1007\/s40291-016-0243-y"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.2340\/00015555-0769"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2016.07.019"},{"key":"e_1_2_7_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jid.2022.09.317"},{"key":"e_1_2_7_24_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.40099"},{"key":"e_1_2_7_25_1","article-title":"Serum analysis of patients with Netherton syndrome shows significant differences compared to other subtypes of ichthyoses: implication for novel therapeutic approaches. (2024), 50th Annual Meeting of the Arbeitsgemeinschaft Dermatologische Forschung (ADF)","volume":"33","author":"S\u00fc\u00dfmuth K","year":"2014","journal-title":"Exp Dermatol"},{"key":"e_1_2_7_26_1","doi-asserted-by":"publisher","DOI":"10.1111\/1346-8138.12410"},{"key":"e_1_2_7_27_1","doi-asserted-by":"publisher","DOI":"10.1084\/jem.20082242"},{"key":"e_1_2_7_28_1","doi-asserted-by":"publisher","DOI":"10.1038\/jid.2010.233"},{"key":"e_1_2_7_29_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2013.08.057"},{"key":"e_1_2_7_30_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.17820"},{"key":"e_1_2_7_31_1","unstructured":"BodesJ PaulK S\u00fc\u00dfmuthK et\u00a0al.Development and Characterization of Recombinant LEKTI Domain 8+9 for Topical Therapy in Netherton Syndrome. Deutsche Pharmazeutische Gesellschaft (Hrsg.):Conference Book \u2013 Pharmaceutical Sciences: Emerging Technologies for Healthy Individuals Annual Meeting of the German Pharmaceutical Society DPhG M\u00fcnster 23\u201326th September 2024:Deutsche Pharmazeutische Gesellschaft 2024 \u2014 ISBN 978\u20103\u20109816225\u20109\u20101."},{"key":"e_1_2_7_32_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jid.2024.04.032"},{"key":"e_1_2_7_33_1","first-page":"405","article-title":"Open\u2010source human skin model with an in vivo\u2010like barrier for drug testing","volume":"39","author":"Zoio P","year":"2022","journal-title":"ALTEX"}],"container-title":["JDDG: Journal der Deutschen Dermatologischen Gesellschaft"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ddg.15928","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,12,11]],"date-time":"2025-12-11T14:13:36Z","timestamp":1765462416000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/ddg.15928"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,10,23]]},"references-count":32,"journal-issue":{"issue":"12","published-print":{"date-parts":[[2025,12]]}},"alternative-id":["10.1111\/ddg.15928"],"URL":"https:\/\/doi.org\/10.1111\/ddg.15928","archive":["Portico"],"relation":{},"ISSN":["1610-0379","1610-0387"],"issn-type":[{"type":"print","value":"1610-0379"},{"type":"electronic","value":"1610-0387"}],"subject":[],"published":{"date-parts":[[2025,10,23]]},"assertion":[{"value":"2024-06-25","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-07-20","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-10-23","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}