1-Deoxynojirimycin (1-DNJ) is a naturally occurring sugar analogue with unique bioactivities.
It is found in mulberry leaves and silkworms, as well as in the metabolites of certain microorganisms,
including Streptomyces and Bacillus. 1-DNJ is a potent α-glucosidase inhibitor and it possesses
anti-hyperglycemic, anti-obese, anti-viral and anti-tumor properties. Some derivatives of 1-DNJ, like
miglitol, miglustat and migalastat, were applied clinically to treat diseases such as diabetes and
lysosomal storage disorders. The present review focused on the extraction, determination, pharmacokinetics
and bioactivity of 1-DNJ, as well as the clinical application of 1-DNJ derivatives.