N-Butyldeoxynojirimycin-d<sub>9</sub> (hydrochloride)

N-Butyldeoxynojirimycin-d9 (hydrochloride)

CAT N°: 34852
Price:

182.00 154.70

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 ?-glucosidase 2 (IC50s = 32 and 81 ?M, respectively, for the rat recombinant enzymes).{38202} It also increases the activity of wild-type acid ?-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 ?M, 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 ?-glucosidase.

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