Etoposide selectively ablates activated T cells to control the immunoregulatory disorder hemophagocytic lymphohistiocytosis

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ID: 120095
2014
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Abstract
Hemophagocytic lymphohistiocytosis (HLH) is an inborn disorder of immune regulation caused by mutations affecting perforin-dependent cytotoxicity. Defects in this pathway impair negative feedback between cytotoxic lymphocytes and APCs, leading to prolonged and pathologic activation of T cells. Etopo …
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Authors Johnson TS;Terrell CE;Millen SH;Katz JD;Hildeman DA;Jordan MB;;
Journal journal of immunology (baltimore, md : 1950)
Year 2014
DOI
DOI not found
URL
http://www.ncbi.nlm.nih.gov/pubmed/24259502
Keywords
National Center for Biotechnology Information NCBI NLM MEDLINE Mice animals pubmed abstract nih national institutes of health national library of medicine research support N.I.H. Extramural Disease Models Animal Apoptosis / drug effects Knockout lymphohistiocytosis etoposide / administration & dosage hemophagocytic / drug therapy* dna-binding proteins / genetics interferon-gamma / biosynthesis t-lymphocytes / immunology* macrophages / drug effects dendritic cells / immunology lymphocyte activation / immunology* apoptosis / immunology pmid:24259502 pmc4177106 doi:10.4049/jimmunol.1302282 theodore s johnson catherine e terrell michael b jordan antigen presentation / drug effects antigen presentation / immunology dendritic cells / drug effects etoposide / pharmacology* lymphocyte activation / drug effects* lymphocytic choriomeningitis virus hemophagocytic / genetics hemophagocytic / immunology* macrophage activation / drug effects macrophage activation / immunology macrophages / immunology t-lymphocytes / drug effects* topoisomerase ii inhibitors / administration & dosage topoisomerase ii inhibitors / pharmacology

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