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Glucosylceramide modulates membrane traffic along the endocytic pathway.

Abstract:

Glycosphingolipids are endocytosed and targeted to the Golgi apparatus, but are mistargeted to lysosomes in numerous sphingolipidoses. Substrate reduction therapy utilizes imino sugars to inhibit glucosylceramide synthase and potentially abrogate the effects of storage. Gaucher disease is a hereditary deficiency in glucocerebrosidase leading to glucosylceramide accumulation; however, Gaucher fibroblasts exhibited normal Golgi transport of lactosylceramide. To better understand the effects of ...

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Publication status:
Published

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Publisher copy:
10.1194/jlr.m200232-jlr200

Authors


Sillence, DJ More by this author
Butters, TD More by this author
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Journal:
Journal of lipid research
Volume:
43
Issue:
11
Pages:
1837-1845
Publication date:
2002-11-05
DOI:
ISSN:
0022-2275
URN:
uuid:fd87b3e9-fb7c-468c-bcc9-ba944a44c27b
Source identifiers:
100966
Local pid:
pubs:100966

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