| The autoreactivity of B cells in hereditary angioedema due to C1 inhibitor deficiency. | |
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MedLine Citation:
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PMID: 22288585 Owner: NLM Status: In-Data-Review |
Abstract/OtherAbstract:
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Patients with hereditary angioedema (HAE) tend to produce autoantibodies and have a propensity to develop immunoregulatory disorders. We characterize the profile of autoantibodies in a group of HAE patients and investigate their memory B cells' phenotype and activation status. We studied the activity status phenotype, Toll-like receptor (TLR)-9 expression and total phosphotyrosine in B cells isolated from HAE patients. Additionally, the following autoantibodies were assessed in the serum of 61 HAE patients: anti-nuclear, rheumatoid factor, anti-cardiolipin, anti-tissue transglutaminase, anti-endomysial, anti-Saccharomyces cerevisiae, anti-thyroid and anti-neutrophil cytoplasmic antibodies. In 47·5% of HAE patients we detected at least one of the tested autoantibodies. Expression of CD69, CD5 and CD21 was found to be significantly higher on memory B cells from HAE patients compared to healthy controls (4·59 ± 4·41 versus 2·06 ± 1·81, P = 0·04, 8·22 ± 7·17 versus 3·65 ± 3·78, P = 0·05, 2·43 ± 0·54 versus 1·92 ± 0·41, P = 0·01, respectively). Total phosphotyrosine in B cells from HAE patients was significantly higher compared to healthy controls (4·8 ± 1·1 versus 2·7 ± 1·3, P = 0·0003). Memory B cells isolated from the HAE group contained higher amounts of TLR-9 compared to healthy controls (8·17 ± 4·1 versus 4·56 ± 1·6, P = 0·0027). Furthermore, the expression of TLR-9 in memory B cells from HAE patients with autoantibodies was significantly higher than the control group (10 ± 4·7 versus 4·56 ± 1·6, P = 0·0002) and from that in HAE patients without autoantibodies (10 ± 4·7 versus 5·8 ± 0·9, P = 0·036). HAE patients have enhanced production of autoantibodies due most probably to the increased activation of B cells, which was found to be in association with a high expression of TLR-9. |
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Authors:
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A Kessel; R Peri; R Perricone; M D Guarino; Z Vadasz; R Novak; T Haj; S Kivity; E Toubi |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Clinical and experimental immunology Volume: 167 ISSN: 1365-2249 ISO Abbreviation: Clin. Exp. Immunol. Publication Date: 2012 Mar |
Date Detail:
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Created Date: 2012-01-31 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 0057202 Medline TA: Clin Exp Immunol Country: England |
Other Details:
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Languages: eng Pagination: 422-8 Citation Subset: IM |
Copyright Information:
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© 2011 The Authors. Clinical and Experimental Immunology © 2011 British Society for Immunology. |
Affiliation:
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Division of Allergy and Clinical Immunology, Bnai Zion Medical Center, Technion Faculty of Medicine, Haifa, Israel Allergy, Clinical Immunology and Rheumatology, University of Rome Tor Vergata, Rome, Italy Allergy and Clinical Immunology Unit, Tel Aviv Medical Center, Sackler School of Medicine, Tel Aviv, Israel. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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