| Restrictive usage of monoclonal immunoglobulin lambda light chain germline in POEMS syndrome. | |
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MedLine Citation:
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PMID: 18497319 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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POEMS syndrome is a rare plasma cell disorder characterized by peripheral neuropathy, monoclonal gammopathy, and high levels of serum vascular endothelial growth factor, the pathogenesis of which remains unclear. A unique feature of this syndrome is that the proliferating monoclonal plasma cells are essentially lambda-restricted. Here we determined complete nucleotide sequences of monoclonal immunoglobulin lambda light chain (IGL) variable regions in 11 patients with POEMS syndrome. The V-region of the Ig lambda gene of all 11 patients was restricted to the V lambda 1 subfamily. Searching for homologies with IGL germlines revealed that 2 germlines, IGLV1-44*01 (9/11) and IGLV1-40*01 (2/10), were identified, with an average homology of 91.1%. The IGLJ3*02 gene was used in 11 of 11 re-arrangements with an average homology of 92.2%. These data suggest that the highly restricted use of IGL V lambda 1 germlines plays an important role in the pathogenesis of POEMS syndrome. |
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Authors:
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Daijiro Abe; Chiaki Nakaseko; Masahiro Takeuchi; Hiroaki Tanaka; Chikako Ohwada; Emiko Sakaida; Yusuke Takeda; Kayo Oda; Shinichi Ozawa; Naomi Shimizu; Shinichi Masuda; Ryuko Cho; Miki Nishimura; Sonoko Misawa; Satoshi Kuwabara; Yasushi Saito |
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Publication Detail:
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Type: Journal Article Date: 2008-05-22 |
Journal Detail:
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Title: Blood Volume: 112 ISSN: 1528-0020 ISO Abbreviation: Blood Publication Date: 2008 Aug |
Date Detail:
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Created Date: 2008-07-24 Completed Date: 2008-09-05 Revised Date: - |
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: 836-9 Citation Subset: AIM; IM |
Affiliation:
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Division of Hematology, Department of Clinical Cell Biology, Chiba University Graduate School of Medicine, Chiba, Japan. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adult Aged Base Sequence Female Germ Cells Humans Immunoglobulin Variable Region / genetics Immunoglobulin lambda-Chains / genetics* Male Middle Aged POEMS Syndrome / etiology* Sequence Homology |
| Chemical | |
Reg. No./Substance:
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0/Immunoglobulin Variable Region; 0/Immunoglobulin lambda-Chains |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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