Article
A novel type of macrothrombocytopenia associated with a defect in α2,3-sialylation.
The American journal of pathology - 1 Oct 2011
Jones Claire, Denecke Jonas, Sträter Ronald, Stölting Torsten, Schunicht Yvonne, Zeuschner Dagmar, Klumperman Judith, Lefeber Dirk J, Spelten Oliver, Zarbock Alexander, Kelm Sørge, Strenge Karen, Haslam Stuart M, Lühn Kerstin, Stahl Dorothea, Gentile Luca, Schreiter Thomas, Hilgard Philip, Beck-Sickinger Annette G, Marquardt Thorsten, Wild Martin K
Abstract excerpt
We describe a novel type of human thrombocytopenia characterized by the appearance of giant platelets and variable neutropenia. Searching for the molecular defect, we found that neutrophils had strongly reduced sialyl-Lewis X and increased Lewis X surface expression, pointing to a deficiency in sialylation. We show that the glycosylation defect is restricted to α2,3-sialylation and can be detected in platelets,...
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