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- Title
Characterization of EBV-transformed B-cells Established from an Individual Homozygously Mutated (G329A) in the FUT7α1,3-fucosyltransferase Gene.
- Authors
Bengtson, P.; Zetterberg, H.; Mellberg, T.; Påhlsson, P.; Larson, G.
- Abstract
The α1,3-fucosyltransferase VII (Fuc-TVII) is involved in the biosynthesis of E- and P-selectin ligands such as sialyl Lewis x (SLex) 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 SLex. 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 SLex-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.
- Subjects
EPSTEIN-Barr virus; FUCOSYLTRANSFERASES; GENES; GENETIC mutation; BIOSYNTHESIS; SELECTINS; CELL adhesion molecules; LEUCOCYTES
- Publication
Scandinavian Journal of Immunology, 2005, Vol 62, Issue 3, p251
- ISSN
0300-9475
- Publication type
Article
- DOI
10.1111/j.1365-3083.2005.01650.x