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Phenotypic differences between mice deficient in XIAP and SAP, two factors targeted in X-linked lymphoproliferative syndrome (XLP).

Publication ,  Journal Article
Rumble, JM; Oetjen, KA; Stein, PL; Schwartzberg, PL; Moore, BB; Duckett, CS
Published in: Cell Immunol
2009

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

Published In

Cell Immunol

DOI

EISSN

1090-2163

Publication Date

2009

Volume

259

Issue

1

Start / End Page

82 / 89

Location

Netherlands

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

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Chicago
ICMJE
MLA
NLM
Rumble, J. M., Oetjen, K. A., Stein, P. L., Schwartzberg, P. L., Moore, B. B., & Duckett, C. S. (2009). Phenotypic differences between mice deficient in XIAP and SAP, two factors targeted in X-linked lymphoproliferative syndrome (XLP). Cell Immunol, 259(1), 82–89. https://doi.org/10.1016/j.cellimm.2009.05.017
Rumble, Julie M., Karolyn A. Oetjen, Paul L. Stein, Pamela L. Schwartzberg, Bethany B. Moore, and Colin S. Duckett. “Phenotypic differences between mice deficient in XIAP and SAP, two factors targeted in X-linked lymphoproliferative syndrome (XLP).Cell Immunol 259, no. 1 (2009): 82–89. https://doi.org/10.1016/j.cellimm.2009.05.017.
Rumble JM, Oetjen KA, Stein PL, Schwartzberg PL, Moore BB, Duckett CS. Phenotypic differences between mice deficient in XIAP and SAP, two factors targeted in X-linked lymphoproliferative syndrome (XLP). Cell Immunol. 2009;259(1):82–9.
Rumble, Julie M., et al. “Phenotypic differences between mice deficient in XIAP and SAP, two factors targeted in X-linked lymphoproliferative syndrome (XLP).Cell Immunol, vol. 259, no. 1, 2009, pp. 82–89. Pubmed, doi:10.1016/j.cellimm.2009.05.017.
Rumble JM, Oetjen KA, Stein PL, Schwartzberg PL, Moore BB, Duckett CS. Phenotypic differences between mice deficient in XIAP and SAP, two factors targeted in X-linked lymphoproliferative syndrome (XLP). Cell Immunol. 2009;259(1):82–89.
Journal cover image

Published In

Cell Immunol

DOI

EISSN

1090-2163

Publication Date

2009

Volume

259

Issue

1

Start / End Page

82 / 89

Location

Netherlands

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