Document Detail

Restrictive usage of monoclonal immunoglobulin lambda light chain germline in POEMS syndrome.
MedLine Citation:
PMID:  18497319     Owner:  NLM     Status:  MEDLINE    
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.
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:
Type:  Journal Article     Date:  2008-05-22
Journal Detail:
Title:  Blood     Volume:  112     ISSN:  1528-0020     ISO Abbreviation:  Blood     Publication Date:  2008 Aug 
Date Detail:
Created Date:  2008-07-24     Completed Date:  2008-09-05     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7603509     Medline TA:  Blood     Country:  United States    
Other Details:
Languages:  eng     Pagination:  836-9     Citation Subset:  AIM; IM    
Division of Hematology, Department of Clinical Cell Biology, Chiba University Graduate School of Medicine, Chiba, Japan.
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MeSH Terms
Base Sequence
Germ Cells
Immunoglobulin Variable Region / genetics
Immunoglobulin lambda-Chains / genetics*
Middle Aged
POEMS Syndrome / etiology*
Sequence Homology
Reg. No./Substance:
0/Immunoglobulin Variable Region; 0/Immunoglobulin lambda-Chains

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