کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
10926989 | 1092292 | 2015 | 6 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
A normal T cell receptor beta CDR3 length distribution in patients with APECED
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کلمات کلیدی
TSATCrAPECEDCDR3mTECAutoimmune polyendocrinopathy–candidiasis–ectodermal dystrophyautoimmune regulator - تنظیم کننده autoimmunesingle positive - تک مثبتThymus - تیموسT cell - سلول تیmedullary thymic epithelial cell - سلول های اپیتلیال تومیک مدولارcomplementarity determining region 3 - منطقه تعریف مکمل 3AIRE - هواT cell receptor - گیرنده سلول T
موضوعات مرتبط
علوم زیستی و بیوفناوری
بیوشیمی، ژنتیک و زیست شناسی مولکولی
بیولوژی سلول
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چکیده انگلیسی
Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is caused by mutations in the AIRE gene. Murine studies suggest that AIRE controls thymic expression of tissue-restricted antigens, its absence allowing nonselected autoreactive cells to escape. We tested this in humans using the TCRβ CDR3 length repertoire as a surrogate of thymic selection, as it shortens during the process. Analysis of healthy thymuses showed an altogether 1.9 base pair shortening, starting at the CD4+CD8+CD3low stage and continuing until the CD4+ subset, likely encompassing both the positive and negative selection. Comparison of five APECED patients with eight healthy controls showed a skewed repertoire with oligoclonal expansions in the patients' CD4+ and CD8+ populations. The average CDR3 length, however, was normal and unaffected by the skewing. This was also true of the hypothesized autoreactive CD8+CD45RA+ population. We failed to detect a subset with an abnormally long CDR3 repertoire, as would be predicted by a failure in selection.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Cellular Immunology - Volume 295, Issue 2, June 2015, Pages 99-104
Journal: Cellular Immunology - Volume 295, Issue 2, June 2015, Pages 99-104
نویسندگان
Heikki J. Niemi, Sini Laakso, Jukka T. Salminen, T. Petteri Arstila, Anni Tuulasvaara,