Jones, Claire, Denecke, Jonas, Straeter, Ronald, Stoelting, Torsten, Schunicht, Yvonne, Zeuschner, Dagmar, Klumperman, Judith, Lefeber, Dirk J., Spelten, Oliver, Zarbock, Alexander, Kelm, Sorge, Strenge, Karen, Haslam, Stuart M., Luehn, Kerstin, Stahl, Dorothea, Gentile, Luca ORCID: 0000-0002-7393-7502, Schreiter, Thomas, Hilgard, Philip, Beck-Sickinger, Annette G., Marquardt, Thorsten and Wild, Martin K. (2011). A Novel Type of Macrothrombocytopenia Associated with a Defect in alpha 2,3-Sialylation. Am. J. Pathol., 179 (4). S. 1969 - 1978. NEW YORK: ELSEVIER SCIENCE INC. ISSN 1525-2191

Full text not available from this repository.

Abstract

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 alpha 2,3-sialylation and can be detected in platelets, neutrophils, and monocytes. Platelets exhibited a distorted structure of the open canalicular system, indicating defective platelet generation. Importantly, patient platelets, but not normal platelets, bound to the asialoglycoprotein receptor (ASGP-R), a liver cell-surface protein that removes desialylated thrombocytes from the circulation in mice. Taken together, this is the first type of human thrombocytopenia in which a specific defect of alpha 2,3-sialylation and an induction of platelet binding to the liver ASGP-R could be detected. (Am J Pathol 2011, 179:1969-1977; DOI: 10.1016/j.ajpath.2011.06.012)

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Jones, ClaireUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Denecke, JonasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Straeter, RonaldUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Stoelting, TorstenUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schunicht, YvonneUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Zeuschner, DagmarUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Klumperman, JudithUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Lefeber, Dirk J.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Spelten, OliverUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Zarbock, AlexanderUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kelm, SorgeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Strenge, KarenUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Haslam, Stuart M.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Luehn, KerstinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Stahl, DorotheaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gentile, LucaUNSPECIFIEDorcid.org/0000-0002-7393-7502UNSPECIFIED
Schreiter, ThomasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hilgard, PhilipUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Beck-Sickinger, Annette G.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Marquardt, ThorstenUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Wild, Martin K.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-487501
DOI: 10.1016/j.ajpath.2011.06.012
Journal or Publication Title: Am. J. Pathol.
Volume: 179
Number: 4
Page Range: S. 1969 - 1978
Date: 2011
Publisher: ELSEVIER SCIENCE INC
Place of Publication: NEW YORK
ISSN: 1525-2191
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
SIALIC-ACID; P-SELECTIN; GLYCOPROTEINS; BIOSYNTHESIS; DEFICIENCY; MEGAKARYOCYTES; PLATELETS; DIAGNOSIS; DISORDER; SURFACEMultiple languages
PathologyMultiple languages
URI: http://kups.ub.uni-koeln.de/id/eprint/48750

Downloads

Downloads per month over past year

Altmetric

Export

Actions (login required)

View Item View Item