{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T10:35:15Z","timestamp":1790073315654,"version":"4.0.1"},"reference-count":25,"publisher":"National Academy of Sciences","issue":"11","content-domain":{"domain":["www.pnas.org"],"crossmark-restriction":true},"short-container-title":["Proc. Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[2009,3,17]]},"abstract":"<jats:p>Patients with autoimmune polyendocrine syndrome type 1 (APS-1) suffer from multiple organ-specific autoimmunity with autoantibodies against target tissue-specific autoantigens. Endocrine and nonendocrine organs such as skin, hair follicles, and liver are targeted by the immune system. Despite sporadic observations of pulmonary symptoms among APS-1 patients, an autoimmune mechanism for pulmonary involvement has not been elucidated. We report here on a subset of APS-1 patients with respiratory symptoms. Eight patients with pulmonary involvement were identified. Severe airway obstruction was found in 4 patients, leading to death in 2. Immunoscreening of a cDNA library using serum samples from a patient with APS-1 and obstructive respiratory symptoms identified a putative potassium channel regulator (KCNRG) as a pulmonary autoantigen. Reactivity to recombinant KCNRG was assessed in 110 APS-1 patients by using immunoprecipitation. Autoantibodies to KCNRG were present in 7 of the 8 patients with respiratory symptoms, but in only 1 of 102 APS-1 patients without respiratory symptoms. Expression of KCNRG messenger RNA and protein was found to be predominantly restricted to the epithelial cells of terminal bronchioles. Autoantibodies to KCNRG, a protein mainly expressed in bronchial epithelium, are strongly associated with pulmonary involvement in APS-1. These findings may facilitate the recognition, diagnosis, characterization, and understanding of the pulmonary manifestations of APS-1.<\/jats:p>","DOI":"10.1073\/pnas.0809986106","type":"journal-article","created":{"date-parts":[[2009,2,26]],"date-time":"2009-02-26T21:45:45Z","timestamp":1235684745000},"page":"4396-4401","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":90,"title":["Pulmonary autoimmunity as a feature of autoimmune polyendocrine syndrome type 1 and identification of KCNRG as a bronchial autoantigen"],"prefix":"10.1073","volume":"106","author":[{"given":"Mohammad","family":"Alimohammadi","sequence":"first","affiliation":[{"name":"Departments of aMedical Sciences and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"No\u00e9mie","family":"Dubois","sequence":"additional","affiliation":[{"name":"Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale, Unit\u00e9 561, Groupe Hospitalier Cochin\u2013Saint Vincent de Paul, F-75674 Paris, France;"},{"name":"Departments of Nutrition, Metabolic Diseases, and Endocrinology, Timone Hospital, Universit\u00e9 de la M\u00e9diterran\u00e9e, 13005 Marseille, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Filip","family":"Sk\u00f6ldberg","sequence":"additional","affiliation":[{"name":"Departments of aMedical Sciences and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u212bsa","family":"Hallgren","sequence":"additional","affiliation":[{"name":"Departments of aMedical Sciences and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isabelle","family":"Tardivel","sequence":"additional","affiliation":[{"name":"Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale, Unit\u00e9 561, Groupe Hospitalier Cochin\u2013Saint Vincent de Paul, F-75674 Paris, 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France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Merja","family":"Kajosaari","sequence":"additional","affiliation":[{"name":"The Hospital for Children and Adolescents, University of Helsinki, Box 281, Fin-00029 HUS, Helsinki, Finland;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William","family":"Egner","sequence":"additional","affiliation":[{"name":"Sheffield Teaching Hospitals National Health Service Foundation Trust, Clinical Immunology and Allergy Unit, Northern General Hospital, Sheffield S5 7AU, United Kingdom;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ravishankar","family":"Sargur","sequence":"additional","affiliation":[{"name":"Sheffield Teaching Hospitals National Health Service Foundation Trust, Clinical Immunology and Allergy Unit, Northern General Hospital, Sheffield S5 7AU, United Kingdom;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fredrik","family":"Pont\u00e9n","sequence":"additional","affiliation":[{"name":"Department of Genetics and Pathology, the Rudbeck Laboratory, Uppsala University, 751 85 Uppsala, Sweden;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zahir","family":"Amoura","sequence":"additional","affiliation":[{"name":"Service de M\u00e9decine Interne, H\u00f4pital Piti\u00e9-Salp\u00eatri\u00e8re and Universit\u00e9 Pierre et Marie Curie Paris 6, 75013 Paris, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alain","family":"Grimfeld","sequence":"additional","affiliation":[{"name":"Department of Pediatric Pulmonology and Allergy, Armand Trousseau Hospital, University Pierre and Marie Curie, 75012 Paris, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Filippo","family":"De Luca","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University 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