| Primary hemophagocytic syndromes point to a direct link between lymphocyte cytotoxicity and homeostasis. | |
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MedLine Citation:
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PMID: 15661029 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Hemophagocytic syndrome (HS) is a severe and often fatal syndrome resulting from potent and uncontrolled activation and proliferation of T-lymphocytes, leading to excessive macrophage activation and multiple deleterious effects. The onset of HS characterizes several inherited disorders in humans. In each condition, the molecular defect impairs the granule-dependent cytotoxic activity of lymphocytes, thus highlighting the determinant role of this function in driving the immune system to a state of equilibrium following infection. It has also been shown that some of the proteins required for lytic granule secretion are required for melanocyte function, leading to associated hypopigmentation in these conditions. This review focuses on several effectors of this secretory pathway, recently identified, because their defects cause these disorders, and discusses their role and molecular interactions in granule-dependent cytotoxic activity. |
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Authors:
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Gael Ménasché; Jérôme Feldmann; Alain Fischer; Geneviève de Saint Basile |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't; Review |
Journal Detail:
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Title: Immunological reviews Volume: 203 ISSN: 0105-2896 ISO Abbreviation: Immunol. Rev. Publication Date: 2005 Feb |
Date Detail:
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Created Date: 2005-01-21 Completed Date: 2005-06-14 Revised Date: 2007-11-15 |
Medline Journal Info:
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Nlm Unique ID: 7702118 Medline TA: Immunol Rev Country: Denmark |
Other Details:
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Languages: eng Pagination: 165-79 Citation Subset: IM |
Affiliation:
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INSERM U429, Hôpital Necker Enfants-Malades, Paris, France. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Cytotoxicity, Immunologic* Histiocytosis, Non-Langerhans-Cell / genetics*, immunology* Homeostasis Humans Melanosomes / physiology Membrane Glycoproteins / genetics, physiology Nerve Tissue Proteins / genetics, physiology Perforin Pore Forming Cytotoxic Proteins Syndrome T-Lymphocytes, Cytotoxic / immunology* rab GTP-Binding Proteins / genetics, physiology |
| Chemical | |
Reg. No./Substance:
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0/Membrane Glycoproteins; 0/Nerve Tissue Proteins; 0/Pore Forming Cytotoxic Proteins; 0/UNC13B protein, human; 126465-35-8/Perforin; EC 3.6.1.-/rab GTP-Binding Proteins; EC 3.6.1.-./RAB27A protein, human |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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