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
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Mentions: To investigate the ability of WAS KO mice to mount a protective immune response against viral infection and to investigate immunopathology, we challenged mice with LCMV (WE strain). Serum alanine aminotransferase and total bilirubin levels were used as a direct measurement of virus-induced hepatic immunopathology.33 WAS KO mice showed a similar, although slightly earlier, alanine aminotransferase response but exhibited increased bilirubin levels after viral challenge (Fig 1, A and B). Furthermore, WAS KO mice showed persistence of viral replication and a hepatic T-cell infiltrate 15 days after infection, whereas by that time, wild-type C57BL/6 animals had cleared viral infection and showed no sign of T-cell infiltration (Fig 1, C and D). These finding indicate that WAS KO mice are compromised in their ability to clear LCMV, despite the presence of an inflammatory T-cell infiltrate.
Affiliation: Campbell Family Institute for Breast Cancer Research, Ontario Cancer Institute, Toronto, Ontario, Canada.