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α-Homonojirimycin
No.125(March 2007)
α-Homonojirimycin (1) is an aza-sugar, whose oxygen atom within the monosaccharide ring is replaced with an imino moiety; it has been shown to significantly affect sugar chain processing in vivo. 1 is an excellent glycosidase inhibitor. The mechanism for its inhibition has been explained as that a glutamic acid or asparagine residue of the glycosidase forms an ionic bond with an imino moiety of the aza-sugar.1) Moreover, 1 is also used as a leading compound of effective agents for the treatment of Fabry disease (disease causing a glyco sphingolipid lysosomal storage disorder), which is caused by an active enzyme deficiency (α-galactosidase A).2)
References
- 1)Glucosidase inhibition of α-Homonojirimycin
- 2)Intracellular enhancement of lysosomal α-Gal A
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