. Author manuscript; available in PMC: 2011 Jun 1.

Published in final edited form as: Semin Immunopathol. 2010 Feb 10;32(2):157–171. doi: 10.1007/s00281-009-0193-0

Table 2.

Disease manifestations of XLP patients and related phenotypes in SAP^−/− mice

Disease manifestations in XLP patients	Observations in SAP^−/− mice
Fatal infectious mononucleosis
Increased numbers of EBV specific CD8+ T cells	Increased numbers of virus specific CTLs and IFN-γ-producing CD8+ cells
Reduced AICD of CD8+ T cells	Decreased apoptosis in CD8+ T cells
Reduced SLAMF-dependent NK cell killing	Reduced SLAMF-dependent NK cell killing
Absence of NKT cells	Absence of NKT cells
B cell lymphomas
Dys-gammaglobulinemia	Impaired primary and secondary antibody responses
Progressive loss of IgG levels and specific antibody responses	Defective Germinal Center formation due to dysfunctional CD4+ ⇔ B cell interactions