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3. Platelet Protein Glycosylation in Immune Thrombocytopenia

Author

Monzón Manzano, Elena, primary, Justo Sanz, Raul, additional, Haslam, Stuart M, additional, Xuan, Leow Ke, additional, Dell, Anne, additional, Álvarez-Roman, Teresa, additional, Martín, Mónica, additional, Rivas Pollmar, María Isabel, additional, Fernandez-Bello, Ihosvany, additional, Canales, Miguel A., additional, Jimenez-Yuste, Victor, additional, and Butta, Nora, additional

Published
2018
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4. Hemoglobin S Induces Exposure of Red Blood Cell Membrane Skeleton Microdomains Bearing Mannose That Stimulate Phagocytosis By Macrophages: A Molecular Basis for Hemolysis in Sickle Cell Disease but Protection Against Plasmodium Falciparum malaria

Author

Cao, Huan, primary, Wassall, Heather J, additional, Forrester, Megan A, additional, Hall, Lindsay S, additional, Wilson, Heather M, additional, Shepherd, Jenna, additional, Patel, Bhinal, additional, Masson, Alanna, additional, Henderson, Sadie, additional, Konieczny, Gabriela, additional, Beverly, Minter, additional, Tampakis, Dimitrios, additional, Antonopoulos, Aristotelis, additional, Haslam, Stuart M, additional, Dell, Anne, additional, Rowe, Alexandra J, additional, Brewin, John, additional, Rees, David C, additional, Barker, Robert N, additional, and Vickers, Mark A, additional

Published
2018
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5. Deficiency Of JAGN1 Causes Severe Congenital Neutropenia Associated With Defective Secretory Pathway and Aberrant Myeloid Cell Homeostasis

Author

Boztug, Kaan, primary, Järvinen, Päivi M, additional, Salzer, Elisabeth, additional, Racek, Tomas, additional, Mönch, Sebastian, additional, Garncarz, Wojciech, additional, Gertz, E. Michael, additional, Schäffer, Alejandro A., additional, Antonopoulos, Aristotelis, additional, Haslam, Stuart, additional, Ziesenitz, Lena, additional, Puchalka, Jacek, additional, Diestelhorst, Jana, additional, Appaswamy, Giridharan, additional, Lescoeur, Brigitte, additional, Giambruno, Roberto, additional, Bigenzahn, Johannes, additional, Elling, Ulrich, additional, Pfeifer, Dietmar, additional, Welte, Karl, additional, Brandes, Gudrun, additional, Sherkat, Roya, additional, Van der Werff Ten Bosch, Jutte, additional, Rezaei, Nima, additional, Etzioni, Amos, additional, Bellanne-Chantelot, Christine, additional, Superti-Furga, Giulio, additional, Bennett, Keiryn L, additional, von Blume, Julia, additional, Dell, Anne, additional, Donadieu, Jean, additional, and Klein, Christoph, additional

Published
2013
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6. Glucose Ceramide Synthase Inhibitors Inhibit Osteoclast Activation Induced by Myeloma-Derived and De Novo Synthesized Glycosphingolipids.

Author

Xu, Ke, primary, Antonopoulos, Aristotelis, additional, Spanoudakis, Emmanouil, additional, Parry, Simon, additional, Chaidos, Aristeidis, additional, Hu, Ming, additional, Butters, Terry, additional, Dell, Anne, additional, Rahemtulla, Amin, additional, Horwood, Nikki, additional, and Karadimitris, Anastasios, additional

Published
2009
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7. Defective Glycosylation of Erythrocyte Membrane Glycoconjugates in a Variant of Congenital Dyserythropoietic Anemia Type II: Association of Low Level of Membrane-Bound Form of Galactosyltransferase

Author

Fukuda, Michiko N., Masri, Khaled A., Dell, Anne, Thonar, Eugene J.-M., Klier, George, and Lowenthal, Ray M.

Abstract

Congenital dyserythropoietic anemia type II (CDA II) or HEMPAS is a genetic disease caused by plasma membrane abnormality. The enzymic defect of HEMPAS has been suggested to be the lowered activity of N-acetylglucosami-nyltransferase II, resulting in lack of polylactosamine formation on proteins and leading to accumulation of polylac-tosaminyl lipids. In contrast to typical HEMPAS cases, cell-surface labeling of the erythrocytes of a HEMPAS variant G.K. showed an absence of polylactosamines either on proteins or on lipids. Fast-atom bombardment mass spectrometry analysis of G.K.'s erythrocyte glycopeptides detected a series of high mannose-type oligosaccharides, which were not detected in erythrocyte N-glycans of normal cells or of other HEMPAS cases: The former con- tains polylactosaminoglycans and the latter contains hybrid-type oligosaccharides. Keratansulfate (sulfated polylactosamines) in this patient's serum was abnormally low. The galactosyltransferase activity in microsomal membranes prepared from G.K.'s mononucleated cells was 24% of the normal level, whereas this enzyme activity in G.K.'s serum was comparatively higher than normal. Western blotting of G.K.'s membranes using antigalactosyl-transferase antibodies showed that G.K. has reduced amounts of this enzyme present. The results collectively suggest that variant G.K. is defective in polylactosamine synthesis owing to the decreased quantity of the mem-brane-bound form of galactosyltransferase.© 1989 by Grune & Stratton, Inc. 0006-4971/89/7305-0011$3.00/0

Published
1989
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