| Defect of plasmacytoid dendritic cells in warts, hypogammaglobulinemia, infections, myelokathexis (WHIM) syndrome patients. | |
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MedLine Citation:
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PMID: 20736454 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Warts, hypogammaglobulinemia, infections, myelokathexis (WHIM) syndrome is a genetic disease that is caused by heterozygous mutations of the CXCR4 gene. These mutations confer an increased leukocyte response to the CXCR4-ligand CXCL12, resulting in abnormal homeostasis of many leukocyte types, including neutrophils and lymphocytes. Analysis of the myeloid and plasmacytoid dendritic cell blood counts in WHIM patients revealed a striking defect in the number of plasmacytoid dendritic cells as well as a partial reduction of the number of myeloid dendritic cells, compared with healthy subjects. Moreover, the production of interferon-α by mononuclear cells in response to herpes simplex infection, or after stimulation with the Toll-like receptor 9 ligand CpG, was undetectable in WHIM patients. Because plasmacytoid dendritic cells play a key role in the defense against viruses and their generation and motility are in part dependent on CXCR4, we hypothesized that the susceptibility of WHIM patients to warts is related to the abnormal homeostasis of plasmacytoid dendritic cells. |
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Authors:
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Laura Tassone; Daniele Moratto; William Vermi; Maria De Francesco; Lucia D Notarangelo; Fulvio Porta; Vassilios Lougaris; Fabio Facchetti; Alessandro Plebani; Raffaele Badolato |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't Date: 2010-08-24 |
Journal Detail:
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Title: Blood Volume: 116 ISSN: 1528-0020 ISO Abbreviation: Blood Publication Date: 2010 Dec |
Date Detail:
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Created Date: 2010-12-03 Completed Date: 2011-01-06 Revised Date: 2011-11-24 |
Medline Journal Info:
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Nlm Unique ID: 7603509 Medline TA: Blood Country: United States |
Other Details:
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Languages: eng Pagination: 4870-3 Citation Subset: AIM; IM |
Affiliation:
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Department of Pediatrics, Institute of Molecular Medicine Angelo Nocivelli, Brescia, Italy. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Cell Separation Dendritic Cells / immunology*, metabolism Flow Cytometry Humans Immunohistochemistry Immunologic Deficiency Syndromes / genetics, immunology, pathology Immunophenotyping Interferon-alpha / biosynthesis* Mutation Receptors, CXCR4 / genetics Warts / genetics, immunology, pathology |
| Grant Support | |
ID/Acronym/Agency:
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GGP07134//Telethon |
| Chemical | |
Reg. No./Substance:
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0/CXCR4 protein, human; 0/Interferon-alpha; 0/Receptors, CXCR4 |
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