Document Detail

Characterization of EBV-transformed B-cells established from an individual homozygously mutated (G329A) in the FUT7 alpha1,3-fucosyltransferase gene.
MedLine Citation:
PMID:  16179012     Owner:  NLM     Status:  MEDLINE    
The alpha1,3-fucosyltransferase VII (Fuc-TVII) is involved in the biosynthesis of E- and P-selectin ligands such as sialyl Lewis x (SLe(x)) on human leukocytes. Recently, individuals were characterized carrying a missense mutation (G329A; Arg110-Gln) in the FUT7 gene encoding this enzyme. The mutated FUT7 construct produced a Fuc-TVII enzyme with impaired activity compared with the wildtype enzyme. Polymorphonuclear granulocytes from an individual carrying this mutation homozygously also showed a reduced expression of SLe(x). In the present study, we have established Epstein-Barr virus-transformed B-cell lines from this individual (SIGN) and from an individual not carrying the mutation (IWO). The cell lines were confirmed to be of B-cell origin by flow cytometry analysis. IWO cells interacted with E-selectin in an in vitro flow chamber analysis whereas SIGN cell did not. However, when SIGN cell was transiently transfected with wildtype FUT7 cDNA, interaction with E-selectin could be restored. Cell surface expression of the SLe(x)-related epitopes recognized by antibodies CSLEX-1, KM-93 and HECA-452 was elevated on IWO cells compared with that on SIGN cells, consistent with a role of these antigens in E-selectin recognition. These cell lines will be useful in further characterization of E-selectin ligands and encourage further studies on the consequences of the FUT7-G329A mutation in vivo.
P Bengtson; H Zetterberg; T Mellberg; P Påhlsson; G Larson
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Scandinavian journal of immunology     Volume:  62     ISSN:  0300-9475     ISO Abbreviation:  Scand. J. Immunol.     Publication Date:  2005 Sep 
Date Detail:
Created Date:  2005-09-23     Completed Date:  2005-11-09     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0323767     Medline TA:  Scand J Immunol     Country:  England    
Other Details:
Languages:  eng     Pagination:  251-8     Citation Subset:  IM    
Department of Biomedicine and Surgery, Division of Cell Biology, Linköping University, Linköping;, Sweden.
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MeSH Terms
B-Lymphocytes / drug effects,  enzymology*,  immunology
Carbohydrates / analysis
Cell Line, Transformed*
DNA, Complementary / genetics
E-Selectin / biosynthesis*,  pharmacology
Epitopes, B-Lymphocyte / analysis
Fucosyltransferases / genetics*
Gene Expression
Herpesvirus 4, Human / physiology*
Mutation, Missense
Oligosaccharides / analysis
RNA, Messenger / analysis,  metabolism
Reg. No./Substance:
0/5-acetylneuraminyl-(2-3)-galactosyl-(1-4)-(fucopyranosyl-(1-3))-N-acetylglucosamine; 0/Carbohydrates; 0/DNA, Complementary; 0/E-Selectin; 0/Epitopes, B-Lymphocyte; 0/Ligands; 0/Oligosaccharides; 0/RNA, Messenger; EC 2.4.1.-/FUT7 protein, human; EC 2.4.1.-/Fucosyltransferases

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