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Therapeutic applications of imino sugars in lysosomal storage disorders.

Abstract:

The N-alkylated imino sugars have inhibitory activity against the first enzyme in the pathway for glucosylating sphingolipid in eukaryotic cells, ceramide-specific glucosyltransferase. A therapeutic approach termed 'substrate deprivation' or 'substrate reduction therapy' (SRT) aims to reduce biosynthetic capability in the cell to match the reduced lysosomal catalytic activity seen in lysosomal storage disorders. The use of N-alkylated imino sugars to establish this therapeutic strategy is des...

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Publication status:
Published

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Publisher copy:
10.2174/1568026033452483

Authors


Butters, TD More by this author
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Institution:
University of Oxford
Department:
Oxford, MSD, Pharmacology
Journal:
Current topics in medicinal chemistry
Volume:
3
Issue:
5
Pages:
561-574
Publication date:
2003
DOI:
EISSN:
1873-4294
ISSN:
1568-0266
URN:
uuid:6329bf88-3694-458b-bb72-531b81d82436
Source identifiers:
100982
Local pid:
pubs:100982

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