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Pulmonary Autoimmun...
Pulmonary Autoimmunity as a Feature of Autoimmune Polyendocrine Syndrome Type 1 and Identification of KCNRG as a Bronchial Autoantigen
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- Alimohammadi, Mohammad (author)
- Uppsala universitet,Medicin,Autoimmuna sjukdomar (Kämpe)
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Dubois, Noemie (author)
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- Sköldberg, Filip (author)
- Uppsala universitet,Institutionen för medicinska vetenskaper,Kolorektalkirurgi,Autoimmuna sjukdomar (Kämpe)
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- Hallgren, Åsa (author)
- Uppsala universitet,Institutionen för medicinska vetenskaper,Autoimmuna sjukdomar (Kämpe)
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Tradivel, Isabelle (author)
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- Hedstrand, Håkan (author)
- Uppsala universitet,Institutionen för medicinska vetenskaper,Autoimmuna sjukdomar (Kämpe)
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Haavik, Jan (author)
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Husebye, Eystein (author)
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- Gustafsson, Jan (author)
- Uppsala universitet,Institutionen för kvinnors och barns hälsa,Barnendokrinologisk forskning/Gustafsson
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- Rorsman, Fredrik (author)
- Uppsala universitet,Institutionen för medicinska vetenskaper,Autoimmuna sjukdomar (Kämpe)
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Meloni, Antonella (author)
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- Janson, Christer (author)
- Uppsala universitet,Medicin,Lungmedicin och allergologi
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Vilattes, Bernard (author)
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Kajosaari, Merja (author)
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Egner, William (author)
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Sargur, Ravishankar (author)
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Amoura, Zahir (author)
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Grimfeld, Alain (author)
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- Pontén, Fredrik (author)
- Uppsala universitet,Molekylär och morfologisk patologi,Hpr
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De Luca, Filippo (author)
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Betterle, Corrado (author)
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Perheentupa, Jaakko (author)
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- Kämpe, Olle (author)
- Uppsala universitet,Medicin,Autoimmuna sjukdomar (Kämpe)
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(creator_code:org_t)
- 2009-03-17
- 2009
- English.
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In: Proceedings of the National Academy of Sciences of the United States of America. - : Proceedings of the National Academy of Sciences. - 0027-8424 .- 1091-6490. ; 106:11, s. 4396-4401
- Related links:
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https://europepmc.or...
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https://urn.kb.se/re...
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https://doi.org/10.1...
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Abstract
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- 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.
Keyword
- MEDICINE
- MEDICIN
Publication and Content Type
- ref (subject category)
- art (subject category)
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- By the author/editor
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Alimohammadi, Mo ...
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Dubois, Noemie
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Sköldberg, Filip
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Hallgren, Åsa
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Tradivel, Isabel ...
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Hedstrand, Håkan
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show more...
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Haavik, Jan
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Husebye, Eystein
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Gustafsson, Jan
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Rorsman, Fredrik
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Meloni, Antonell ...
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Janson, Christer
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Vilattes, Bernar ...
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Kajosaari, Merja
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Egner, William
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Sargur, Ravishan ...
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Amoura, Zahir
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Grimfeld, Alain
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Pontén, Fredrik
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De Luca, Filippo
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Betterle, Corrad ...
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Perheentupa, Jaa ...
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Kämpe, Olle
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Proceedings of t ...
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Uppsala University