| Mechanisms of disease: aquaporin-4 antibodies in neuromyelitis optica. | |
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MedLine Citation:
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PMID: 18334978 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Neuromyelitis optica (NMO) is a rare CNS inflammatory disorder that predominantly affects the optic nerves and spinal cord. Recent serological findings strongly suggest that NMO is a distinct disease rather than a subtype of multiple sclerosis. In NMO, serum antibodies, collectively known as NMO-IgG, characteristically bind to cerebral microvessels, pia mater and Virchow-Robin spaces. The main target antigen for this immunoreactivity has been identified as aquaporin-4 (AQP4). The antibodies are highly specific for NMO, and they are also found in patients with longitudinally extensive transverse myelitis without optic neuritis, which is thought to be a precursor to NMO in some cases. An antibody-mediated pathogenesis for NMO is supported by several observations, including the characteristics of the AQP4 antibodies, the distinct NMO pathology--which includes IgG and complement deposition and loss of AQP4 from spinal cord lesions--and emerging evidence of the beneficial effects of B-cell depletion and plasma exchange. Many aspects of the pathogenesis, however, remain unclear. |
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Authors:
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Sven Jarius; Friedemann Paul; Diego Franciotta; Patrick Waters; Frauke Zipp; Reinhard Hohlfeld; Angela Vincent; Brigitte Wildemann |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't; Review Date: 2008-03-11 |
Journal Detail:
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Title: Nature clinical practice. Neurology Volume: 4 ISSN: 1745-8358 ISO Abbreviation: - Publication Date: 2008 Apr |
Date Detail:
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Created Date: 2008-04-04 Completed Date: 2008-08-04 Revised Date: 2008-10-02 |
Medline Journal Info:
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Nlm Unique ID: 101261799 Medline TA: Nat Clin Pract Neurol Country: England |
Other Details:
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Languages: eng Pagination: 202-14 Citation Subset: IM |
Affiliation:
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Weatherall Institute of Molecular Medicine, University of Oxford, UK. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Antibodies
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blood* Aquaporin 4 / immunology*, metabolism Binding Sites, Antibody* Humans Neuromyelitis Optica / drug therapy, immunology*, pathology |
| Chemical | |
Reg. No./Substance:
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0/Antibodies; 0/Aquaporin 4; 0/Binding Sites, Antibody |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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