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Journal article

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

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Journal:
Journal of lipid research More from this journal
Volume:
43
Issue:
11
Pages:
1837-1845
Publication date:
2002-11-01
DOI:
ISSN:
0022-2275
Language:
English
Keywords:
Pubs id:
pubs:100966
UUID:
uuid:fd87b3e9-fb7c-468c-bcc9-ba944a44c27b
Local pid:
pubs:100966
Source identifiers:
100966
Deposit date:
2012-12-19

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