Shelf life (days): 1460.0
Formulation: A solid
Formal Name: 1-butyl-d9-2R-(hydroxymethyl)-3R,4R,5S-piperidinetriol, monohydrochloride
Purity: ≥99% deuterated forms (d1-d9)
Formula Markup: C10H12D9NO4 / HCl
Formula Weight: 264.79326
CAS Number: 1883545-57-0
Notes: N-Butyldeoxynojirimycin-d9 (NB-DNJ-d9) is intended for use as an internal standard for the quantification of NB-DNJ (Item No. 21065) by GC- or LC-MS. NB-DNJ is an iminosugar that inhibits UDP-glucose ceramide glucosyltransferase and β-glucosidase 2 (IC50s = 32 and 81 μM, respectively, for the rat recombinant enzymes).{38202} It also increases the activity of wild-type acid β-glucosidase, as well as the S364R, N370S, V15M, or M123T mutants, in COS-7 cells expressing the human enzymes when used at a concentration of 10 µM.{38203} NB-DNJ also inhibits HIV-1 and HIV-2 infection of peripheral blood mononuclear cells (PBMCs; IC50s = 282 and 211 μM, respectively).{38204} Formulations containing NB-DNJ have been used in the treatment of Gaucher disease, an inborn error of metabolism characterized as a lysosomal storage disorder resulting from substantial deficiency of β-glucosidase.
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