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Patients with X-Linked Lymphoproliferative Syndrome Type 1 (XLP1) Have Mutations $\gamma$

question 23

Multiple Choice

Patients with X-linked lymphoproliferative syndrome type 1 (XLP1) have mutations in the SLAM-family adapter protein, SAP. These patients suffer from an inability to control infections of the herpesvirus Epstein-Barr virus (EBV) that infects B cells. Studies have been done using peripheral blood T cells from these patients to examine their response to EBV in comparison to the responses of normal individuals (EBV-infected) as controls. These data were obtained by ELISPOT analysis, to quantify the numbers of IFN- $\gamma$ -producing CD8 T cells per million peripheral blood leukocytes in response to several EBV peptides, indicated as peptide 1, 2, 3, or 4 (note: the patients and controls were matched for HLA class I molecules) .
$Patients with X-linked lymphoproliferative syndrome type 1 (XLP1) have mutations in the SLAM-family adapter protein, SAP. These patients suffer from an inability to control infections of the herpesvirus Epstein-Barr virus (EBV) that infects B cells. Studies have been done using peripheral blood T cells from these patients to examine their response to EBV in comparison to the responses of normal individuals (EBV-infected) as controls. These data were obtained by ELISPOT analysis, to quantify the numbers of IFN- \gamma -producing CD8 T cells per million peripheral blood leukocytes in response to several EBV peptides, indicated as peptide 1, 2, 3, or 4 (note: the patients and controls were matched for HLA class I molecules) . These results indicate that: A) XLP patients have a defect in priming CD8 T cell responses following EBV infection. B) XLP patients require IFN- \gamma production to kill EBV-infected B cells. C) XLP patients have normal CD8 effector cell priming following EBV infection. D) XLP patients have a defect in cytotoxicity by NK cells rather than by CD8 T cells. E) XLP patients have no defect in cytotoxicity by CD8 T cells.$
These results indicate that:

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Patients with X-Linked Lymphoproliferative Syndrome Type 1 (XLP1) Have Mutations γ\gammaγ

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Patients with X-Linked Lymphoproliferative Syndrome Type 1 (XLP1) Have Mutations $\gamma$