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ShareScienceDaily (Sep. 14, 2009) — X-linked lymphoproliferative disease (XLP) is a rare inherited immunodeficiency most commonly caused by deficiency in the protein SAP. Following infection with the common virus that causes infectious mononucleosis (also known as mono or glandular fever), boys with XLP often develop an extreme, usually fatal, accumulation of activated immune cells known as cytotoxic T lymphocytes; but the mechanistic link between this and SAP deficiency has not been determined.
However, Michael Lenardo and colleagues, at the National Institutes of Health in Bethesda, Md., have now found that T cells from individuals with XLP are resistant to cell death triggered by repeated stimulation of a cell surface protein complex known as the TCR.
They report their research in the Journal of Clinical Investigation.
As repeated TCR stimulation normally constrains T cell expansion during immune responses, the authors propose that this makes the T cells susceptible to uncontrolled expansion upon infection.
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The above story is reprinted from materials provided by Journal of Clinical Investigation, via EurekAlert!, a service of AAAS.
Note: Materials may be edited for content and length. For further information, please contact the source cited above.
Journal Reference:
- Snow et al. Restimulation-induced apoptosis of T cells is impaired in patients with X-linked lymphoproliferative disease caused by SAP deficiency. Journal of Clinical Investigation, 2009; DOI: 10.1172/JCI39518
Note: If no author is given, the source is cited instead.
Disclaimer: This article is not intended to provide medical advice, diagnosis or treatment. Views expressed here do not necessarily reflect those of ScienceDaily or its staff.
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