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What Happens When Immune Cells Just Won't Die?

Date:
September 17, 2009
Source:
Journal of Clinical Investigation
Summary:
X-linked lymphoproliferative disease (XLP) is a rare inherited immunodeficiency most commonly caused by deficiency in the protein SAP. New research now provides an explanation as to how SAP deficiency causes boys with XLP to develop an extreme, usually fatal, accumulation of activated immune cells known as cytotoxic T lymphocytes following infection with the common virus that causes mono.
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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.


Story Source:

The above post is reprinted from materials provided by Journal of Clinical Investigation. Note: Materials may be edited for content and length.


Journal Reference:

  1. 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

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Journal of Clinical Investigation. "What Happens When Immune Cells Just Won't Die?." ScienceDaily. ScienceDaily, 17 September 2009. <www.sciencedaily.com/releases/2009/09/090914172334.htm>.
Journal of Clinical Investigation. (2009, September 17). What Happens When Immune Cells Just Won't Die?. ScienceDaily. Retrieved July 30, 2015 from www.sciencedaily.com/releases/2009/09/090914172334.htm
Journal of Clinical Investigation. "What Happens When Immune Cells Just Won't Die?." ScienceDaily. www.sciencedaily.com/releases/2009/09/090914172334.htm (accessed July 30, 2015).

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