Article
A Fluorescent sp2-iminosugar with pharmacological chaperone activity for gaucher disease: synthesis and intracellular distribution studies.
Chembiochem : a European journal of chemical biology - 22 Nov 2010
Luan Zhuo, Higaki Katsumi, Aguilar-Moncayo Matilde, Li Linjing, Ninomiya Haruaki, Nanba Eiji, Ohno Kousaku, García-Moreno M Isabel, Ortiz Mellet Carmen, García Fernández José M, Suzuki Yoshiyuki
Abstract excerpt
Gaucher disease (GD) is the most prevalent lysosomal-storage disorder, it is caused by mutations of acid β-glucosidase (β-glucocerebrosidase; β-Glu). Recently, we found that bicyclic nojirimycin (NJ) derivatives of the sp(2)-iminosugar type, including the 6-thio-N'-octyl-(5N,6S)-octyliminomethyli...
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