General Information of Drug (ID: DM2ATZB)

Drug Name
Deoxynojirimycin Drug Info
Synonyms
1-DEOXYNOJIRIMYCIN; 19130-96-2; DUVOGLUSTAT; deoxynojirimycin; Moranoline; (2R,3R,4R,5S)-2-(hydroxymethyl)piperidine-3,4,5-triol; 1,5-Deoxy-1,5-imino-D-mannitol; Moranolin; UNII-FZ56898FLE; D-1-deoxynojirimycin; 1,5-Dideoxy-1,5-imino-D-glucitol; CHEMBL307429; C6H13NO4; FZ56898FLE; CHEBI:44369; 1-Deoxy-Nojirimycin; 5-Amino-1,5-dideoxy-D-glucopyranose; DNJ; AK151410; 3,4,5-Piperidinetriol, 2-(hydroxymethyl)-, (2R,3R,4R,5S)-; 3,4,5-Piperidinetriol, 2-(hydroxymethyl)-, (2R-(2alpha,3beta,4alpha,5beta))-
Indication
Disease Entry ICD 11 Status REF
Pompe disease 5C51.3 Phase 3 [1]
Cross-matching ID
PubChem CID
29435
ChEBI ID
CHEBI:44369
CAS Number
CAS 19130-96-2
TTD Drug ID
DM2ATZB

Molecule(s) Related to This Drug


Drug Therapeutic Target (DTT)
DTT Name DTT ID UniProt ID MOA REF
Alpha-glucosidase (GLA) TTHCF4J LYAG_HUMAN; GANAB_HUMAN; GANC_HUMAN Inhibitor [2]
Intestinal maltase-glucoamylase (MGAM) TTXWASR MGA_HUMAN Inhibitor [2]
Lysosomal alpha-glucosidase (GAA) TTLPC70 LYAG_HUMAN Inhibitor [3]

The Expression Level of Molecule(s) between Disease Section and Healthy Individual Tissue

The Studied Disease Pompe disease
ICD Disease Classification 5C51.3
Molecule Name Molecule Type Gene Name p-value Fold-Change Z-score
Intestinal maltase-glucoamylase (MGAM) DTT MGAM 4.59E-01 -0.02 -0.24
Molecular Expression Atlas between Disease Section and Healthy Individual Tissue

References

1 The pharmacological chaperone 1-deoxynojirimycin increases the activity and lysosomal trafficking of multiple mutant forms of acid alpha-glucosidase. Hum Mutat. 2009 Dec;30(12):1683-92.
2 Nitrogen-in-the-ring pyranoses and furanoses: structural basis of inhibition of mammalian glycosidases. J Med Chem. 1994 Oct 28;37(22):3701-6.
3 In vitro inhibition of glycogen-degrading enzymes and glycosidases by six-membered sugar mimics and their evaluation in cell cultures. Bioorg Med Chem. 2008 Aug 1;16(15):7330-6.