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Autoantibodies Targeting a Collecting Duct-Specific Water Channel in Tubulointerstitial Nephritis

Landegren, Nils (author)
Uppsala universitet,Karolinska Institutet,Uppsala University,Karolinska Institute,Karolinska University Hospital,Science for Life Laboratory, SciLifeLab,Autoimmunitet,Karolinska Inst, Karolinska Univ Hosp, Dept Med Solna, SE-17176 Stockholm, Sweden
Pourmousa Lindberg, Mina (author)
Uppsala universitet,Uppsala University,Institutionen för medicinska vetenskaper
Skov, Jakob (author)
Uppsala universitet,Karolinska Institutet,Uppsala University,Karolinska Institute,Karolinska University Hospital,Science for Life Laboratory, SciLifeLab,Karolinska Inst, Karolinska Univ Hosp, Dept Med Solna, SE-17176 Stockholm, Sweden
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Hallgren, Åsa (author)
Uppsala universitet,Uppsala University,Karolinska Institute,Karolinska University Hospital,Autoimmunitet,Science for Life Laboratory, SciLifeLab,Karolinska Inst, Karolinska Univ Hosp, Dept Med Solna, SE-17176 Stockholm, Sweden
Eriksson, Daniel (author)
Uppsala universitet,Karolinska Institutet,Uppsala University,Karolinska Institute,Karolinska University Hospital,Science for Life Laboratory, SciLifeLab,Institutionen för medicinska vetenskaper,Karolinska Inst, Karolinska Univ Hosp, Dept Med Solna, SE-17176 Stockholm, Sweden
Lisberg Toft-Bertelsen, Trine (author)
University of Copenhagen,Univ Copenhagen, Dept Cellular & Mol Med, Copenhagen, Denmark
MacAulay, Nanna (author)
University of Copenhagen,Univ Copenhagen, Dept Cellular & Mol Med, Copenhagen, Denmark
Hagforsen, Eva (author)
Uppsala universitet,Uppsala University,Dermatologi och venereologi
Räisänen-Sokolowski, Anne (author)
University of Helsinki,Helsinki University Central Hospital,Helsinki Univ Hosp, Dept Pathol, Helsinki, Finland.; Univ Helsinki, Helsinki, Finland
Saha, Heikki (author)
University of Tampere School of Medicine,Tampere Univ Hosp, Sch Med, Dept Med, Tampere, Finland
Nilsson, Thomas (author)
Uppsala universitet,Uppsala University,Institutionen för medicinska vetenskaper
Nordmark, Gunnel (author)
Uppsala universitet,Uppsala University,Reumatologi
Ohlsson, Sophie (author)
Lund University,Lunds universitet,Njurmedicin,Sektion II,Institutionen för kliniska vetenskaper, Lund,Medicinska fakulteten,Autoimmunitet och njursjukdomar,Forskargrupper vid Lunds universitet,Nephrology,Section II,Department of Clinical Sciences, Lund,Faculty of Medicine,Autoimmunity and kidney diseases,Lund University Research Groups,Lund Univ, Dept Nephrol, Lund, Sweden
Gustafsson, Jan (author)
Uppsala universitet,Uppsala University,Pediatrik
Husebye, Eystein S (author)
University of Bergen,Haukeland University Hospital,Karolinska Inst, Karolinska Univ Hosp, Dept Med Solna, SE-17176 Stockholm, Sweden.; Univ Bergen, Dept Clin Sci, Bergen, Norway.; Haukeland Hosp, Dept Med, Bergen, Norway
Larsson, Erik (author)
Uppsala universitet,Uppsala University,Institutionen för immunologi, genetik och patologi
Anderson, Mark S (author)
University of California System,Univ Calif San Francisco, Ctr Diabet, San Francisco, CA 94143 USA
Perheentupa, Jaakko (author)
University of Helsinki,Univ Helsinki, Hosp Children & Adolescents, Helsinki, Finland
Rorsman, Fredrik (author)
Uppsala universitet,Uppsala University,Gastroenterologi/hepatologi
Fenton, Robert A (author)
Aarhus University,Aarhus Univ, Dept Biomed, Interact Prot Epithelial Transport Ctr, Aarhus, Denmark
Kämpe, Olle (author)
Uppsala universitet,Karolinska Institutet,Uppsala University,Karolinska Institute,Karolinska University Hospital,Autoimmunitet,Karolinska Inst, Karolinska Univ Hosp, Dept Med Solna, SE-17176 Stockholm, Sweden
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 (creator_code:org_t)
2016
2016
English 9 s.
In: Journal of the American Society of Nephrology: JASN. - 1533-3450 .- 1046-6673. ; 27:10, s. 3220-3228
  • Journal article (peer-reviewed)
Abstract Subject headings
Close  
  • Tubulointerstitial nephritis is a common cause of kidney failure and may have diverse etiologies. This form of nephritis is sometimes associated with autoimmune disease, but the role of autoimmune mechanisms in disease development is not well understood. Here, we present the cases of three patients with autoimmune polyendocrine syndrome type 1 who developed tubulointerstitial nephritis and ESRD in association with autoantibodies against kidney collecting duct cells. One of the patients developed autoantibodies targeting the collecting duct-specific water channel aquaporin 2, whereas autoantibodies of the two other patients reacted against the HOXB7 or NFAT5 transcription factors, which regulate the aquaporin 2 promoter. Our findings suggest that tubulointerstitial nephritis developed in these patients as a result of an autoimmune insult on the kidney collecting duct cells.

Subject headings

MEDICIN OCH HÄLSOVETENSKAP  -- Klinisk medicin -- Urologi och njurmedicin (hsv//swe)
MEDICAL AND HEALTH SCIENCES  -- Clinical Medicine -- Urology and Nephrology (hsv//eng)
MEDICIN OCH HÄLSOVETENSKAP  -- Klinisk medicin -- Reumatologi och inflammation (hsv//swe)
MEDICAL AND HEALTH SCIENCES  -- Clinical Medicine -- Rheumatology and Autoimmunity (hsv//eng)

Keyword

Adult
Aquaporins/immunology
Autoantibodies/immunology
Female
Humans
Kidney Tubules, Collecting/immunology
Male
Middle Aged
Nephritis, Interstitial/immunology
Young Adult
Medical Science

Publication and Content Type

art (subject category)
ref (subject category)

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