Crystal structures of complexes of N-butyl- and N-nonyl-deoxynojirimycin bound to acid beta-glucosidase: insights into the mechanism of chemical chaperone action in Gaucher disease.

Gaucher disease is caused by mutations in the gene encoding acid beta-glucosidase (GlcCerase), resulting in glucosylceramide (GlcCer) accumulation. The only currently available orally administered treatment for Gaucher disease is N-butyl-deoxynojirimycin (Zavesca, NB-DNJ), which partially inhibits G...

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Detaylı Bibliyografya
Asıl Yazarlar: Brumshtein, B, Greenblatt, H, Butters, T, Shaaltiel, Y, Aviezer, D, Silman, I, Futerman, A, Sussman, J
Materyal Türü: Journal article
Dil:English
Baskı/Yayın Bilgisi: 2007