Research ArticleImmunodeficiency

Inhibition of diacylglycerol kinase α restores restimulation-induced cell death and reduces immunopathology in XLP-1

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Science Translational Medicine  13 Jan 2016:
Vol. 8, Issue 321, pp. 321ra7
DOI: 10.1126/scitranslmed.aad1565

SAPping immunopathology

Individuals with deficient immune systems may also paradoxically experience hyperimmune side effects. X-linked lymphoproliferative disease (XLP-1)—an immunodeficiency caused by defects in the T cell receptor adaptor protein SAP [signaling lymphocytic activation molecule (SLAM)–associated protein]—is associated with expansion of activated T cell after viral infection. Now, Ruffo et al. report that down-regulating diacylglycerol kinase α (DGKα) in SAP-deficient T cells restores restimulation-induced cell death, preventing this excess expansion. If these data hold true in humans, targeting DGKα may prevent viral-induced immunopathology in XLP-1 patients.

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