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Aire deficient mice...
Aire deficient mice develop multiple features of APECED phenotype and showaltered immune response
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Ramsey, C (author)
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- Winqvist, Ola (author)
- Uppsala universitet,Ludwiginstitutet för cancerforskning
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Puhakka, L (author)
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Halonen, M (author)
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Moro, A (author)
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- Kämpe, Olle (author)
- Uppsala universitet,Ludwiginstitutet för cancerforskning
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Eskelin, P (author)
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Pelto-Huikko, M (author)
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Peltonen, L (author)
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(creator_code:org_t)
- Oxford University Press (OUP), 2002
- 2002
- English.
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In: Human Molecular Genetics. - : Oxford University Press (OUP). - 0964-6906 .- 1460-2083. ; 11:4, s. 397-409
- Related links:
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https://academic.oup...
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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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- Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is a monogenic autosomal recessive disease caused by mutations in the AIRE gene. Here we have produced knock-out mice for the Aire gene. The Aire-/- mice develop normally; however, autoimmune features of APECED in Aire-/- mice are evident, including multiorgan lymphocytic infiltration, circulating autoantibodies and infertility. The distribution of B and T cells and thymic maturation as well as activation of T cells appear normal, while the TCR-Vbeta repertoire is altered in peripheral T cells of Aire-/- mice. When mice are challenged with immunization, the peripheral T cells of Aire-/- mice have a 3-5-fold increased proliferation. These findings suggest that the Aire gene is not necessary for normal T cell education and development, while a defect in immune response detected in challenged Aire-/- mice underlines the crucial role of AIRE/Aire in maintaining homeostatic regulation in the immune system.
Publication and Content Type
- ref (subject category)
- art (subject category)
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