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Invariant natural killer T cells are not affected by lysosomal storage in patients with Niemann-Pick disease type C.

Abstract:

Invariant natural killer T (iNKT) cells are a specialised subset of T cells that are restricted to the MHC class I like molecule, CD1d. The ligands for iNKT cells are lipids, with the canonical superagonist being α-galactosylceramide, a non-mammalian glycosphingolipid. Trafficking of CD1d through the lysosome is required for the development of murine iNKT cells. Niemann-Pick type C (NPC) disease is a lysosomal storage disorder caused by dysfunction in either of two lysosomal proteins, NPC1 or...

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Publication status:
Published

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Publisher copy:
10.1002/eji.201141821

Authors


te Vruchte, D More by this author
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Journal:
European journal of immunology
Volume:
42
Issue:
7
Pages:
1886-1892
Publication date:
2012-07-05
DOI:
EISSN:
1521-4141
ISSN:
0014-2980
URN:
uuid:b6756729-99b9-4b1c-b10d-21b8b0601d0b
Source identifiers:
332772
Local pid:
pubs:332772

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