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Blood, 15 August 2008, Vol. 112, No. 4, pp. 1472-1481. Prepublished online as a Blood First Edition Paper on June 9, 2008; DOI 10.1182/blood-2008-01-132035. This Article Full Text (PDF) Supplemental Figure All Versions of this Article: blood-2008-01-132035v1 112/4/1472    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Yakubenia, S. Articles by Wild, M. K. Search for Related Content PubMed PubMed Citation Articles by Yakubenia, S. Articles by Wild, M. K. Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted January 9, 2008 Accepted May 27, 2008 Leukocyte trafficking in a mouse model for Leukocyte Adhesion Deficiency II / Congenital Disorder of Glycosylation IIc Sviatlana Yakubenia, David Frommhold, Dirk Scholch, Christina C. Hellbusch, Christian Korner, Bjorn Petri, Claire Jones, Ute Ipe, M. Gabriele Bixel, Robert Krempien, Markus Sperandio, and Martin K. Wild* Vascular Cell Biology, Max Planck Institute for Molecular Biomedicine, Muenster, Germany Department of Pediatrics, Section of Neonatology, University Children's Hospital, Heidelberg, Heidelberg, Germany Department of Pediatrics, Division of Inborn Metabolic Diseases, University Children's Hospital, Heidelberg, Heidelberg, Germany Department of Radiation Oncology, University Heidelberg, Heidelberg, Germany Walter Brendel Center of Experimental Medicine, Ludwig-Maximilians-University, Muenchen, Germany * Corresponding author; email: mwild{at}mpi-muenster.mpg.de. Leukocyte Adhesion Deficiency II (LAD II), also termed Congenital Disorder of Glycosylation IIc (CDG-IIc), is a human disease in which a defective GDP-fucose transporter (SLC35C1) causes developmental defects and an immunodeficiency which is based on the lack of fucosylated selectin ligands. Since the study of in vivo leukocyte trafficking in LAD II patients is experimentally limited we analyzed this process in mice deficient for Slc35c1. We found that E-, L-, and P-selectin-dependent leukocyte rolling in cremaster muscle venules was virtually absent. This was accompanied by a strong, but not complete, decrease in firm leukocyte adhesion. Moreover, neutrophil migration to the inflamed peritoneum was strongly reduced by 89%. Previous reports showed surprisingly normal lymphocyte functions in LAD II which indicated sufficient lymphocyte trafficking to secondary lymphoid organs. We now found that while lymphocyte homing to lymph nodes was reduced to 1-2% in Slc35c1-/- mice, trafficking to the spleen was completely normal. In accordance with this we found a defect in the humoral response to a T cell-dependent antigen in lymph nodes but not in the spleen. Taken together, Slc35c1-/- mice show strongly defective leukocyte trafficking but normal lymphocyte homing to the spleen which may explain normal lymphocyte functions in LAD II. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: L. D. Notarangelo and R. Badolato Leukocyte trafficking in primary immunodeficiencies J. Leukoc. Biol., March 1, 2009; 85(3): 335 - 343. [Abstract] [Full Text] [PDF]

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