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Article

Therapeutic Applications of Imino Sugars in Lysosomal Storage Disorders

1 Feb 2003

Abstract excerpt

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....

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PMID: 12570866
DOI: 10.2174/1568026033452483

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