The Journal of Experimental Medicine
Aegean Conferences: 2009 Conferences
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Published online 25 September 2006 doi:10.1084/jem.20061787
Rockefeller University Press, 0022-1007 $8.00
JEM, Volume 203, Number 10, 2229-2232
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COMMENTARY

 Chewing the fat on natural killer T cell development

Dale I. Godfrey, Malcolm J. McConville, and Daniel G. Pellicci

CORRESPONDENCE D.I.G.: godfrey{at}unimelb.edu.au


ABSTRACT
Natural killer T cells (NKT cells) are selected in the thymus by self-glycolipid antigens presented by CD1d molecules. It is currently thought that one specific component of the lysosomal processing pathway, which leads to the production of isoglobotrihexosylceramide (iGb3), is essential for normal NKT cell development. New evidence now shows that NKT cell development can be disrupted by a diverse range of mutations that interfere with different elements of the lysosomal processing and degradation of glycolipids. This suggests that lysosomal storage diseases (LSDs) in general, rather than one specific defect, can disrupt CD1d antigen presentation, leading to impaired development of NKT cells.

D.I.G. and D.G.P are at the Department of Microbiology and Immunology, University of Melbourne, and M.J.M. is at the Department of Biochemistry and Molecular Biology, Bio21 Molecular Science and Biotechnology Institute, University of Melbourne, Victoria 3010, Australia.


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J. Exp. Med. 2006 203: 2293-2303. [Abstract] [Full Text] [PDF]



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