DEOXY-NOJIRIMYCIN DERIVATIVE AND USE OF DERIVATIVE AS GLUCOSYLCERAMIDASE INHIBITOR

摘要

PROBLEM TO BE SOLVED: To provide a method and a pharmaceutical composition for treating a disease involved in a signal transmission system through ceramide, for example, a disease selected from the group consisting of gaucher disease, tay-sachs disease, sandhoff disease and niemann-pick disease.SOLUTION: A deoxy-nojirimycin derivative or a salt thereof contains a large hydrophobic part such as adamantane methanol and cholesterol binding to a nitrogen atom of deoxy-nojirimycin through a spacer, for example, pentamethylene and inhibits glucosylceramide.