{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T03:46:20Z","timestamp":1790826380009,"version":"4.1.0"},"reference-count":41,"publisher":"Oxford University Press (OUP)","issue":"1","license":[{"start":{"date-parts":[[2006,7,1]],"date-time":"2006-07-01T00:00:00Z","timestamp":1151712000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2006,7,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Niemann-Pick Type C1 (NPC1) is a late endosomal\/lysosomal transmembrane protein involved in the cellular transport of glycosphingolipids and cholesterol that is mutated in a majority of patients with Niemann-Pick C neurodegenerative disease. We found that NPC1-deficient mice lacked V\u03b114-J\u03b118 NKT cells, a major population of CD1d-restricted T cells that is conserved in humans. NPC1-deficient mice also exhibited marked defects in the presentation of Sphingomonas cell wall Ags to NKT cells and in bacterial clearance in vivo. A synthetic fluorescent \u03b1-glycosylceramide analog of the Sphingomonas Ag trafficked to the lysosome of wild-type cells but accumulated in the late endosome of NPC1-deficient cells. These findings reveal a blockade of lipid trafficking between endosome and lysosome as a consequence of NPC1 deficiency and suggest a common mechanism for the defects in lipid presentation and development of V\u03b114-J\u03b118 NKT cells.<\/jats:p>","DOI":"10.4049\/jimmunol.177.1.26","type":"journal-article","created":{"date-parts":[[2014,4,18]],"date-time":"2014-04-18T19:47:14Z","timestamp":1397850434000},"page":"26-30","source":"Crossref","is-referenced-by-count":70,"title":["Cutting Edge: Impaired Glycosphingolipid Trafficking and NKT Cell Development in Mice Lacking Niemann-Pick Type C1 Protein"],"prefix":"10.1093","volume":"177","author":[{"given":"Yuval","family":"Sagiv","sequence":"first","affiliation":[{"name":"Committee on Immunology, University of Chicago , Chicago, IL 60637"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kelly","family":"Hudspeth","sequence":"additional","affiliation":[{"name":"Committee on Immunology, University of Chicago , Chicago, IL 60637"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jochen","family":"Mattner","sequence":"additional","affiliation":[{"name":"Committee on Immunology, University of Chicago , Chicago, IL 60637"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicolas","family":"Schrantz","sequence":"additional","affiliation":[{"name":"Department of Immunology, The Scripps Research Institute , La Jolla, CA 92037"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Randi K","family":"Stern","sequence":"additional","affiliation":[{"name":"Committee on Immunology, University of Chicago , Chicago, IL 60637"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dapeng","family":"Zhou","sequence":"additional","affiliation":[{"name":"MD Anderson Cancer Center, University of Texas , Houston, TX 77030"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul B","family":"Savage","sequence":"additional","affiliation":[{"name":"Department of Chemistry, Brigham Young University , Provo UT 84602"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luc","family":"Teyton","sequence":"additional","affiliation":[{"name":"Department of Immunology, The Scripps Research Institute , La Jolla, CA 92037"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Albert","family":"Bendelac","sequence":"additional","affiliation":[{"name":"Committee on Immunology, University of Chicago , Chicago, IL 60637"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2006,7,1]]},"reference":[{"key":"2026020210142532600_R1","first-page":"175","volume-title":"Mol. 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