Reduced type I interferon production by dendritic cells and weakened antiviral immunity in patients with Wiskott-Aldrich syndrome protein deficiency.
Bottom Line: Despite a significant proportion of patients with WAS having recurrent viral infections, surprisingly little is known about the effects of WASP deficiency on antiviral immunity.IFN-I production by WAS KO DCs was reduced both in vivo and in vitro.These findings might help us to understand the immunodeficiency of WAS.
Affiliation: Campbell Family Institute for Breast Cancer Research, Ontario Cancer Institute, Toronto, Ontario, Canada.Show MeSH
Related in: MedlinePlus
Mentions: To analyze the CD8+ response in vivo in more detail, we infected wild-type C57BL/6 or WAS KO mice with LCMV and analyzed the virus-specific CD8+ T-cell response. Six days after infection, the total number of CD8+ T cells and LCMV-specific, GP33 tetramer–positive CD8+ T cells in the spleen, liver, and blood was similar between C57BL/6 and WAS KO mice (Fig 3, A-C). However, on days 12 and 20 after infection, the numbers of total and virus-specific CD8+ T cells recovered from blood was markedly reduced in WAS KO mice (Fig 3, C), whereas at that time, LCMV had been eliminated in C57BL/6 mice (Fig 1, C). WAS KO mice also mounted CD8+ T-cell responses specific for the immunodominant epitope of the LCMV nucleoprotein (NP396), but although reduced compared with values seen in C57BL/6 mice, this did not reach statistical significance (see Fig E1 in this article's Online Repository at www.jacionline.org). A typical CD8+ T-cell response will peak around day 8 after infection, after which only a small subset of CD8+ T cells will survive and develop into memory T cells. This subset can be identified by IL-7 receptor (IL-7R) expression.39,41,42
Affiliation: Campbell Family Institute for Breast Cancer Research, Ontario Cancer Institute, Toronto, Ontario, Canada.