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Ann Allergy Asthma Immunol. 2010 Apr;104(4):286-92. doi: 10.1016/j.anai.2010.01.021.

Underexpression and overexpression of Fas and Fas ligand: a double-edged sword.

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  • 1Department of Pediatrics, Section of Allergy and Immunology, LSU Health Sciences Center, Shreveport, Louisiana 71130, USA.

Abstract

OBJECTIVE:

To compare autoimmune lymphoproliferative syndrome (ALPS) and Stevens-Johnson syndrome (SJS) with respect to the defects in Fas- and Fas ligand (FasL)-mediated apoptosis.

DATA SOURCES:

Selected reviews, case reports, and original studies were searched in PubMed and MEDLINE for the keywords ALPS, SJS, Fas, FasL, and apoptosis.

STUDY SELECTION:

Case reports of ALPS and SJS were selected as examples of Fas- and FasL-mediated diseases. In addition, we selected articles that examined the pathophysiology of apoptosis in the context of Fas-FasL interaction.

RESULTS:

Failure to initiate apoptosis of abnormal T lymphocytes occurs in such diseases as ALPS, leading to the accumulation of double negative T cells with an increase in autoimmunity. In contrast to apoptotic failure, SJS is associated with a pathological increase in programmed keratinocyte cell death.

CONCLUSION:

The consequences of dysregulated Fas- and FasL-mediated apoptosis leads to self-reactivity, malignant transformation, and immune dysfunction. An understanding of underlying mechanisms and qualitative assessment of Fas and FasL may have clinical benefits when control of these homeostatic mechanisms is in question.

PMID:
20408337
[PubMed - indexed for MEDLINE]
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