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Altered expression of autoimmune regulator in infant down syndrome thymus, a possible contributor to an autoimmune phenotype.

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Authors
Skogberg, Gabriel
Lundberg, Vanja
Lindgren, Susanne
Gudmundsdottir, Judith
Sandström, Kerstin
Kämpe, Olle
Annerén, Göran
Gustafsson, Jan
Sunnegårdh, Jan
van der Post, Sjoerd
Telemo, Esbjörn
Berglund, Martin
Ekwall, Olov
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Issue Date
2014-09-01

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Citation
J. Immunol. 2014, 193 (5):2187-95
Abstract
Down syndrome (DS), caused by trisomy of chromosome 21, is associated with immunological dysfunctions such as increased frequency of infections and autoimmune diseases. Patients with DS share clinical features, such as autoimmune manifestations and specific autoantibodies, with patients affected by autoimmune polyendocrine syndrome type 1. Autoimmune polyendocrine syndrome type 1 is caused by mutations in the autoimmune regulator (AIRE) gene, located on chromosome 21, which regulates the expression of tissue-restricted Ags (TRAs) in thymic epithelial cells. We investigated the expression of AIRE and TRAs in DS and control thymic tissue using quantitative PCR. AIRE mRNA levels were elevated in thymic tissue from DS patients, and trends toward increased expression of the AIRE-controlled genes INSULIN and CHRNA1 were found. Immunohistochemical stainings showed altered cell composition and architecture of the thymic medulla in DS individuals with increased frequencies of AIRE-positive medullary epithelial cells and CD11c-positive dendritic cells as well as enlarged Hassall's corpuscles. In addition, we evaluated the proteomic profile of thymic exosomes in DS individuals and controls. DS exosomes carried a broader protein pool and also a larger pool of unique TRAs compared with control exosomes. In conclusion, the increased AIRE gene dose in DS could contribute to an autoimmune phenotype through multiple AIRE-mediated effects on homeostasis and function of thymic epithelial cells that affect thymic selection processes.
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Additional Links
http://dx.doi.org/ 10.4049/jimmunol.1400742
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4135177/
http://www.jimmunol.org/content/193/5/2187.full.pdf
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Archived with thanks to Journal of immunology (Baltimore, Md. : 1950)
ae974a485f413a2113503eed53cd6c53
10.4049/jimmunol.1400742
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English Journal Articles (Peer Reviewed)

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