Perforin-mediated target-cell death and immune homeostasis View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

2006-12

AUTHORS

Ilia Voskoboinik, Mark J. Smyth, Joseph A. Trapani

ABSTRACT

The granule exocytosis pathway of cytotoxic lymphocytes is crucial for immune surveillance and homeostasis. The trafficking of granule components, including the membrane-disruptive protein perforin, to the immunological synapse leads to the delivery of granule proteases (granzymes) into the target cell and its destruction through apoptosis. Several independent molecular abnormalities associated with defects of either granule trafficking or perforin function can cause cytotoxic lymphocyte dysfunction. In humans, inherited perforin mutations result in severe immune dysregulation that manifests as familial haemophagocytic lymphohistiocytosis. This Review describes recent progress in defining the structure, function, biochemistry and cell biology of perforin. More... »

PAGES

940-952

References to SciGraph publications

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  • Identifiers

    URI

    http://scigraph.springernature.com/pub.10.1038/nri1983

    DOI

    http://dx.doi.org/10.1038/nri1983

    DIMENSIONS

    https://app.dimensions.ai/details/publication/pub.1026188939

    PUBMED

    https://www.ncbi.nlm.nih.gov/pubmed/17124515


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