| Inherited defects causing hemophagocytic lymphohistiocytic syndrome. | |
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MedLine Citation:
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PMID: 22236431 Owner: NLM Status: In-Data-Review |
Abstract/OtherAbstract:
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Hemophagocytic lymphohistiocytosis (HLH) manifests as the uncontrolled activation of T lymphocytes and macrophages infiltrating multiple organs. Molecular studies of individuals with HLH have demonstrated in most of these conditions a critical role of granule-dependent cytotoxic activity in the regulation of lymphocyte homeostasis, and have allowed the characterization of key effectors regulating cytotoxic granule release. The cytolytic process may now be considered a multistep process, including cell activation; the polarization of cytotoxic granules toward the conjugated target cell; the tethering, priming, and fusion of the cytotoxic granules with the plasma membrane; and the release of their contents (perforin and granzymes) into the intercellular cleft, leading to target cell death. Cytolytic cells have a second effector function involving the production of cytokines, principally γ-interferon, which is secreted independently of the exocytosis cytotoxic granule pathway. An analysis of the mechanisms underlying HLH has identified γ-interferon as a key cytokine inducing uncontrolled macrophage activation, and thus represents a potential therapeutic target. |
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Authors:
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Geneviève de Saint Basile; Gaël Ménasché; Sylvain Latour |
Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Annals of the New York Academy of Sciences Volume: 1246 ISSN: 1749-6632 ISO Abbreviation: Ann. N. Y. Acad. Sci. Publication Date: 2011 Dec |
Date Detail:
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Created Date: 2012-01-12 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 7506858 Medline TA: Ann N Y Acad Sci Country: United States |
Other Details:
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Languages: eng Pagination: 64-76 Citation Subset: IM |
Copyright Information:
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© 2011 New York Academy of Sciences. |
Affiliation:
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Institut National de la Santé et de la Recherche Médicale INSERM, U768, Paris, France. Université Paris Descartes, Sorbone Paris Cité, Faculté de Médecine, Necker Hospital, 75015 Paris, France. Centre d'Etudes des Déficits Immunitaires, Assistance Publique-Hôpitaux de Paris (AP-HP) Hôpital Necker, 75015 Paris, France. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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