title: Chewing the fat on natural killer T cell development.
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.
authors.affiliation: Department of Microbiology and Immunology, University of Melbourne, Victoria, Australia. godfrey@unimelb.edu.au
authors.forename: Dale I
authors.initials: DI
authors.lastname: Godfrey
authors.forename: Malcolm J
authors.initials: MJ
authors.lastname: McConville
authors.forename: Daniel G
authors.initials: DG
authors.lastname: Pellicci
mesh_terms: D000818:Animals; D018949:Antigens, CD1 / Q000276:immunology; D055610:Antigens, CD1d; D002454:Cell Differentiation / Q000276:immunology*; D005915:Globosides / Q000096:biosynthesis*; D006017:Glycolipids / Q000276:immunology; D006801:Humans; D007694:Killer Cells, Natural / Q000166:cytology*; D016464:Lysosomal Storage Diseases / Q000276:immunology*; D051379:Mice; D018448:Models, Immunological; D016176:T-Lymphocyte Subsets / Q000166:cytology*; D014281:Trihexosylceramides / Q000096:biosynthesis*
chemical_list: D018949:Antigens, CD1; D055610:Antigens, CD1d; C526877:CD1D protein, human; D005915:Globosides; D006017:Glycolipids; D014281:Trihexosylceramides; C514781:isoglobotrihexosylceramide