Chemical Properties
White Crystalline Solid
Definition
ChEBI: N-nonyldeoxynojirimycin is a hydroxypiperidine that is deoxynojirimycin (duvoglustat) in which the amino hydrogen is replaced by a nonyl group. It has a role as an EC 3.2.1.45 (glucosylceramidase) inhibitor, an EC 3.2.1.20 (alpha-glucosidase) inhibitor and an antiviral agent. It is a hydroxypiperidine and a tertiary amino compound. It is functionally related to a duvoglustat.
Biological Activity
Glucosidase inhibitor (IC 50 values are 0.42 and 8.4 μ M for acid α -glucosidase and α -1,6-glucosidase respectively). Inhibits liver glycogen breakdown in vivo . Also acts as a chemical chaperone; chaperones β -Glu folding at neutral p.H. allowing the stabilized enzyme to transit from the endoplasmic reticulum to the golgi, enabling proper trafficking to the lysosome.
Biochem/physiol Actions
NN-DNJ is a glucosidase inhibitor that acts as a pharmacologic chaperone. NN-DNJ also acts synergistically with proteostasis modulators such as celastrol and MG132 to stabilize mutant proteins in properly folded states. NN-DNJ stabilizes various proteins against misfolding, increasing proper trafficking from the endoplasmic reticulum. There is potential use in studying diseases marked by misfolded proteins and has shown antiviral activity.
