
Phenotypic differences between mice deficient in XIAP and SAP, two factors targeted in X-linked lymphoproliferative syndrome (XLP).
Mutations in the X-linked inhibitor of apoptosis (XIAP) have recently been identified in patients with the rare genetic disease, X-linked lymphoproliferative syndrome (XLP), which was previously thought to be solely attributable to mutations in a distinct gene, SAP. To further understand the roles of these two factors in the pathogenesis of XLP, we have compared mice deficient in Xiap with known phenotypes of Sap-null mice. We show here that in contrast to Sap-deficient mice, animals lacking Xiap have apparently normal NKT cell development and no apparent defect in humoral responses to T cell-dependent antigens. However, Xiap-deficient cells were more susceptible to death upon infection with the murine herpesvirus MHV-68 and gave rise to more infectious virus. These differences could be rescued by restoration of XIAP. These data provide insight into the differing roles of XIAP and SAP in the pathogenesis of XLP.
Duke Scholars
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Related Subject Headings
- X-Linked Inhibitor of Apoptosis Protein
- Signaling Lymphocytic Activation Molecule Associated Protein
- Natural Killer T-Cells
- Mice, Knockout
- Mice, Inbred C57BL
- Mice
- Lymphoproliferative Disorders
- Intracellular Signaling Peptides and Proteins
- Immunology
- Herpesviridae Infections
Citation

Published In
DOI
EISSN
Publication Date
Volume
Issue
Start / End Page
Location
Related Subject Headings
- X-Linked Inhibitor of Apoptosis Protein
- Signaling Lymphocytic Activation Molecule Associated Protein
- Natural Killer T-Cells
- Mice, Knockout
- Mice, Inbred C57BL
- Mice
- Lymphoproliferative Disorders
- Intracellular Signaling Peptides and Proteins
- Immunology
- Herpesviridae Infections