Migalastat

drug
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Also known as At-1001gr-181314aGalafoldSID11114003

Summary

Migalastat (CHEMBL110458) is an approved small molecule (ATC A16AX14) targeting GAA; indicated across 1 condition including fabry disease.

At a glance

  • Status: Approved (max clinical phase 4)
  • Modality: Small molecule
  • ATC class: A16AX14
  • Targets: 1 (GAA)
  • Indications: 1 condition
  • Clinical trials: 9
  • Chemistry: 163.17 Da · C6H13NO4

Identifiers

Drug identity and classification

FieldValue
ChEMBL IDCHEMBL110458
NameMigalastat
TypeSmall molecule
Max phase4
FDA approvedyes
PubChem CID176077
ATCA16AX14
Molecular formulaC6H13NO4
Molecular weight163.17
InChIKeyLXBIFEVIBLOUGU-DPYQTVNSSA-N

SMILES: C1[C@@H]([C@H]([C@H]([C@H](N1)CO)O)O)O

IUPAC name: (2R,3S,4R,5S)-2-(hydroxymethyl)piperidine-3,4,5-triol

Also known as: At-1001, gr-181314a, Galafold, Migalastat, SID11114003, MIGALASTAT, migalastat

Parent form; salt/anhydrous children: CHEMBL2107355

Patent coverage: 139 distinct patent families (430 SureChEMBL compound mentions), from 1 matched compound structure(s). Mentions count patents naming the compound (not distinct inventions), so promiscuous / reference molecules inflate the mention figure — families are the dedup metric.

Targets

Targets

Primary targets (GtoPdb curated mechanism): the Cancer dependency column is the DepMap CRISPR fitness signal (% of screened cell lines dependent on the target).

GeneTargetActionpAffinityCancer dependencyUniProt
GAAalpha glucosidaseBinding7.40.4%P10253

Broader ChEMBL bioactivity targets: 16 (assay-derived). Sample: Lactase/phlorizin hydrolase, Beta-galactosidase, Maltase-glucoamylase, Menin/Histone-lysine N-methyltransferase MLL, Lysosomal acid glucosylceramidase, Beta-galactosidase, Alpha-galactosidase A, Lysosomal alpha-glucosidase, Sucrase-isomaltase, intestinal, Polyunsaturated fatty acid lipoxygenase ALOX15.

Bioactivity

ChEMBL activities: 15 potent at pChembl ≥ 5 of 23 total. Top 30 by potency (10 = 0.1 nM, 6 = 1 µM):

TargetpChemblTypeValueUnitActivity ID
Q426568.52IC503nMCHEMBL_ACT_3342719
GLA7.89IC5013nMCHEMBL_ACT_18001636
Q426567.89Ki13nMCHEMBL_ACT_3353523
GLA7.38IC5042nMCHEMBL_ACT_18001643
GLA7.16IC5070nMCHEMBL_ACT_1405782
GLA7.16IC5070nMCHEMBL_ACT_3342720
GAA7.08Potency82.3nMCHEMBL_ACT_4898651
SI6.58IC50260nMCHEMBL_ACT_3204035
GBA15.85Potency1422nMCHEMBL_ACT_4741462
GBA15.85Potency1422nMCHEMBL_ACT_4793382
MGAM5.82IC501500nMCHEMBL_ACT_3204048
Q9UUZ45.75IC501800nMCHEMBL_ACT_1405791
GAA5.22IC506000nMCHEMBL_ACT_3204099
AGL5IC5010000nMCHEMBL_ACT_3204111
GAA5Potency10000nMCHEMBL_ACT_4403882

Target pathways

Aggregated over 1 target gene(s): GAA.

Top Reactome pathways

12 total, by targets touching each:

PathwayTargetsGenes
Metabolism1GAA
Disease1GAA
Innate Immune System1GAA
Immune System1GAA
Glycogen storage diseases1GAA
Glycogen storage disease type II (GAA)1GAA
Diseases of carbohydrate metabolism1GAA
Diseases of metabolism1GAA
Neutrophil degranulation1GAA
Glycogen breakdown (glycogenolysis)1GAA
Metabolism of carbohydrates and carbohydrate derivatives1GAA
Glycogen metabolism1GAA

Dominant GO biological processes

GO termTargets
maltose metabolic process1
regulation of the force of heart contraction1
diaphragm contraction1
heart morphogenesis1
glycogen catabolic process1
sucrose metabolic process1
glucose metabolic process1
lysosome organization1
locomotory behavior1
tissue development1
aorta development1
obsolete vacuolar sequestering1
muscle cell cellular homeostasis1
neuromuscular process controlling posture1
neuromuscular process controlling balance1

Indications & clinical

Indications

1 indication (0 at ChEMBL trial phase 4). Phase below is the highest clinical-trial phase recorded for this drug against each disease — not the molecule’s overall approval status (that is in the Summary).

IndicationTrial phaseMONDOEFO
Fabry disease3MONDO:0010526MONDO:0010526

Clinical trials

Total trials: 9.

Phase distribution

PhaseTrials
PHASE36
Not specified3

Top trials by phase / activity

NCTPhaseStatusTitle
NCT04020055PHASE3ACTIVE_NOT_RECRUITINGA Study to Evaluate Migalastat in Fabry Subjects With Amenable GLA Variant and Renal Disease
NCT05280548PHASE3ACTIVE_NOT_RECRUITINGA Study to Evaluate the Effect of Venglustat Tablets on Left Ventricular Mass Index in Male and Female Adult Participants With Fabry Disease
NCT06904261PHASE3RECRUITINGA Study of Migalastat in Pediatric Subjects (2 to <12 Yrs) With Fabry Disease and Amenable GLA Variants
NCT02194985PHASE3COMPLETEDOpen-Label Extension Study of the Long-Term Effects of Migalastat HCL in Patients With Fabry Disease
NCT03500094PHASE3COMPLETEDSafety, Pharmacokinetics, Pharmacodynamics, and Efficacy of Migalastat in Pediatric Subjects (Aged 12 to <18 Years)
NCT04049760PHASE3COMPLETEDSafety, Pharmacodynamics, and Efficacy of Migalastat in Pediatric Subjects (Aged >12 Years) With Fabry Disease
NCT01476163Not specifiedAVAILABLEPhysician Initiated Expanded Access Request for Migalastat in Individual Patients With Fabry Disease
NCT04252066Not specifiedRECRUITINGA Global Prospective Observational Study of Women With Fabry Disease and Their Infants During Pregnancy and Breastfeeding
NCT03949920Not specifiedUNKNOWNA Study of Migalastat in Fabry Disease

Clinical evidence (CIViC)

No CIViC predictive evidence (expected for non-precision-medicine drugs).

Pharmacology

Pharmacogenomics

No CPIC/DPWG dosing guideline, but PharmGKB curates 19 clinical and 20 variant annotation(s) for this drug (gene-keyed; see PharmGKB).

Molecules sharing ≥1 of this drug’s curated primary targets, merged from two biobtree sources and ranked by shared-target count, then clinical phase: ChEMBL clinical-stage candidates (development phase ≥2) and PubChem drug-class bioactivity (approved / known drugs acting on the target). Deduplicated by drug name; the drug’s own salt forms are excluded. Note: for a drug with few primary targets a shared-target match can reflect off-target / promiscuous binding rather than the same therapeutic mechanism — the phase ordering surfaces bona-fide therapeutics first.

108 molecules share ≥1 primary target. Top 60 by shared-target count:

MoleculeSourceStatusShared targets
ACARBOSEChEMBLPhase 4 (approved)GAA
ACRISORCINChEMBLPhase 4 (approved)GAA
ALFUZOSINChEMBLPhase 4 (approved)GAA
AMILORIDEChEMBLPhase 4 (approved)GAA
AMLEXANOXChEMBLPhase 4 (approved)GAA
APOMORPHINEChEMBLPhase 4 (approved)GAA
ARIPIPRAZOLEChEMBLPhase 4 (approved)GAA
ASTEMIZOLEChEMBLPhase 4 (approved)GAA
BETAXOLOLChEMBLPhase 4 (approved)GAA
CARBARILChEMBLPhase 4 (approved)GAA
CARVEDILOLChEMBLPhase 4 (approved)GAA
CHLORPROMAZINEChEMBLPhase 4 (approved)GAA
CHLORTETRACYCLINEChEMBLPhase 4 (approved)GAA
CINNARIZINEChEMBLPhase 4 (approved)GAA
DEFEROXAMINEChEMBLPhase 4 (approved)GAA
DEMECLOCYCLINEChEMBLPhase 4 (approved)GAA
DIBUCAINEChEMBLPhase 4 (approved)GAA
DICLOFENACChEMBLPhase 4 (approved)GAA
DIENESTROLChEMBLPhase 4 (approved)GAA
DIPYRIDAMOLEChEMBLPhase 4 (approved)GAA
DITHIAZANINE IODIDEChEMBLPhase 4 (approved)GAA
DOPAMINEChEMBLPhase 4 (approved)GAA
DOXAZOSINChEMBLPhase 4 (approved)GAA
DULOXETINEChEMBLPhase 4 (approved)GAA
ECONAZOLE NITRATEChEMBLPhase 4 (approved)GAA
EDARAVONEChEMBLPhase 4 (approved)GAA
EPINEPHRINE BITARTRATEChEMBLPhase 4 (approved)GAA
FELBINACChEMBLPhase 4 (approved)GAA
FLUOROMETHOLONEChEMBLPhase 4 (approved)GAA
FLUSPIRILENEChEMBLPhase 4 (approved)GAA
FOLIC ACIDChEMBLPhase 4 (approved)GAA
INDAPAMIDEChEMBLPhase 4 (approved)GAA
INDOPROFENChEMBLPhase 4 (approved)GAA
IRBESARTANChEMBLPhase 4 (approved)GAA
ISOETHARINEChEMBLPhase 4 (approved)GAA
ISOPROTERENOLChEMBLPhase 4 (approved)GAA
LABETALOLChEMBLPhase 4 (approved)GAA
LANSOPRAZOLEChEMBLPhase 4 (approved)GAA
MAPROTILINEChEMBLPhase 4 (approved)GAA
MECLOFENAMATEChEMBLPhase 4 (approved)GAA
MENADIONEChEMBLPhase 4 (approved)GAA
METHYLDOPAChEMBLPhase 4 (approved)GAA
METHYSERGIDEChEMBLPhase 4 (approved)GAA
MICONAZOLE NITRATEChEMBLPhase 4 (approved)GAA
MIGLITOLChEMBLPhase 4 (approved)GAA
MIGLUSTATChEMBLPhase 4 (approved)GAA
MORICIZINEChEMBLPhase 4 (approved)GAA
NIACINChEMBLPhase 4 (approved)GAA
OLANZAPINEChEMBLPhase 4 (approved)GAA
OXYTETRACYCLINEChEMBLPhase 4 (approved)GAA
PHENELZINEChEMBLPhase 4 (approved)GAA
PHENYLEPHRINEChEMBLPhase 4 (approved)GAA
PRAZOSINChEMBLPhase 4 (approved)GAA
PRILOCAINEChEMBLPhase 4 (approved)GAA
PROCHLORPERAZINEChEMBLPhase 4 (approved)GAA
QUINESTROLChEMBLPhase 4 (approved)GAA
RABEPRAZOLEChEMBLPhase 4 (approved)GAA
RILUZOLEChEMBLPhase 4 (approved)GAA
TERAZOSINChEMBLPhase 4 (approved)GAA
TERFENADINEChEMBLPhase 4 (approved)GAA