l-NBDNJ

L-NBDNJ (N-butyl-l-deoxynojirimycin) is the L-enantiomer of the iminosugar N-butyl-deoxynojirimycin (NB-DNJ, also known as miglustat), synthetically derived from L-glucose. It is a small molecule that serves as an allosteric enhancer of lysosomal α-glucosidase activity, investigated primarily for its pharmacological chaperone therapy potential in diseases such as Pompe disease. Unlike the D-enantiomer, which acts as a glycosidase inhibitor, L-NBDNJ does not significantly inhibit glycosidases, but rather can increase the activity and cellular stability of α-glucosidase. L-NBDNJ also shows anti-inflammatory and anti-infective actions in preclinical models, including effects relevant to cystic fibrosis and chronic Pseudomonas aeruginosa infection[1][2][3][7].

Other names

N-butyl-l-deoxynojirimycinL-NBDNJ hydrochloride