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  • Open Access

Abundant CD4+FOXP3+ regulatory T cells fail to suppress the proliferation of T cells in patients with Turner syndrome

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International Journal of Pediatric Endocrinology20152015 (Suppl 1) :O25

  • Published:


  • Public Health
  • Internal Medicine
  • Fluorescence Intensity
  • Autoimmune Disease
  • Mononuclear Cell


Why Turner syndrome (TS) patients are predisposed to autoimmune disease remains unclear.


We investigated whether the frequency, phenotype, and suppressive function of CD4+FOXP3+ regulatory T cells (Tregs) are altered in young TS patients with the 45,X karyotype compared to age-matched controls.


Peripheral blood mononuclear cells from young TS patients (n = 24, 17.4–35.9 yrs) and controls (n = 29) were stained with various Treg markers to characterize their phenotypes. Tregs sorted for CD4+CD25bright were co-cultured with autologous CD4+CD25 target cells in the presence of anti-CD3 and CD28 antibodies to assess their suppressive function.


TS patients exhibited a higher frequency of CD4+FOXP3+ Tregs among their lymphocytes (mean 2.06 vs. 1.52%, P = 0.005) and FOXP3+ Tregs among their CD4+ T cells (7.44 vs. 4.19%, P < 0.001) compared to controls. The expression of inhibitory CTLA-4 in the Tregs of TS patients was also significantly higher (mean fluorescence intensity = 214.1 vs. 184.6, P = 0.003). The frequency of Tregs expressing GITR+, CXCR3+, and CCR4+CCR6+ was comparable between the two groups. However, the ability of Tregs to suppress the in vitro proliferation of autologous CD4+CD25 T cells was significantly impaired in TS patients compared to controls (P < 0.05 at a 0.1:1 ratio of Tregs to target cells, P < 0.01 at 0.25:1, 0.5:1, and 1:1).


The Tregs of TS patients could not efficiently suppress the proliferation of autologous effector T cells, despite the abundance of Tregs in the peripheral circulation.

Authors’ Affiliations

Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea
Department of Anatomy, Seoul National University College of Medicine, Seoul, Korea
Department of Biomedical Sciences, Seoul National University College of Medicine, Seoul, Korea
Department of Pathology, Seoul National University Hospital, Seoul, Korea
Ischemic/Hypoxia Institute, Seoul National University College of Medicine, Seoul, Korea


© Ah Lee et al; licensee BioMed Central Ltd. 2015

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