Article
Chaperone activity of bicyclic nojirimycin analogues for Gaucher mutations in comparison with N-(n-nonyl)deoxynojirimycin.
Chembiochem : a European journal of chemical biology - 23 Nov 2009
Luan Zhuo, Higaki Katsumi, Aguilar-Moncayo Matilde, Ninomiya Haruaki, Ohno Kousaku, García-Moreno M Isabel, Ortiz Mellet Carmen, García Fernández José M, Suzuki Yoshiyuki
Abstract excerpt
Gaucher disease (GD), the most prevalent lysosomal storage disorder, is caused by mutations of lysosomal beta-glucosidase (acid beta-Glu, beta-glucocerebrosidase); these mutations result in protein misfolding. Some inhibitors of this enzyme, such as the iminosugar glucomimetic N-(n-nonyl)-1-deoxynojirimycin (NN-DNJ), are known to bind to the active site and stabilize the proper folding for the catalytic form,...
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