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NCT00214500

A Study of AT1001 (Migalastat Hydrochloride) in Participants With Fabry Disease

Completed Phase 2 Results posted Last updated 30 October 2018
What this trial tests

Phase 2 trial testing migalastat HCl in Fabry Disease in 9 participants. Completed in 29 January 2008.

Timeline
2 January 2006
Primary endpoint
29 January 2008
29 January 2008

Quick facts

Lead sponsorAmicus Therapeutics
PhasePhase 2
StatusCompleted
Study typeINTERVENTIONAL
Allocationna
Designsingle group
Maskingnone
Primary purposetreatment
Enrollment9
Start date2 January 2006
Primary completion29 January 2008
Estimated completion29 January 2008
Sites5 locations across United States

Drugs / interventions tested

Conditions studied

Sponsor

Amicus Therapeutics — full company profile →

Who can join

Adults 18 to 55, male only, with Fabry Disease. Patients with the condition only — healthy volunteers not accepted.

Results — posted to ClinicalTrials.gov

Per-arm endpoint measurements with 95% confidence intervals where reported. Source: trial results section.

Number Of Participants Who Experienced Severe Treatment-emergent Adverse Events (TEAEs) Primary · Day 1 (after dosing) through Week 96

TEAEs were defined as any adverse event with start date on or after administration of study drug or pre-existing conditions that worsened on or after the start of the first study drug administration (on Day 1). A severe adverse event was defined as an adverse event that was incapacitating and required medical intervention. The number of participants who experienced one or more severe TEAEs after dosing on Day 1 through Week 96 is presented. A summary of serious and all other non-serious adverse events regardless of causality is located in the Reported Adverse Events module.

GroupValue95% CI
Migalastat2
PK: Area Under The Concentration Versus Time Curve (AUC) After Administration Of Migalastat Secondary · 0 (predose), 0.5, 1, 2, 3, 4, 5, 6, 8, and 10 hr (postdose)

The AUC from time zero to 12 hours (hr) postdose (AUC0-12) was evaluated in plasma following a single dose of migalastat 25, 100, and 250 mg on Days 1, 15, and 29, respectively. In addition, AUC0-12 was assessed following multiple doses (14 days) of migalastat 25, 100, and 250 mg on Days 14, 28, and 42, respectively.

AUC0-12: Single Dose
GroupValue95% CI
Migalastat 25 mg1052.96± 29.9
Migalastat 100 mg4217.95± 32.0
Migalastat 250 mg10880.66± 32.4
AUC0-12: Multiple Dose
GroupValue95% CI
Migalastat 25 mg1360.69± 32.9
Migalastat 100 mg5643.50± 25.3
Migalastat 250 mg12244.47± 26.0
α-Galactosidase A (α-Gal A) Activity In Leukocytes At Baseline, Week 12, And Week 96 Secondary · Baseline, Week 12 (end of treatment period), Week 96 (end of extension period)

Leukocytes were isolated from whole blood and lysed, and α-Gal A activity was measured using a validated fluorometric assay, with catalysis to fluorescent 4-methylumbelliferone (4-MU) as the activity measure. The activity values obtained were normalized to protein (measured using a colorimetric assay) and reported as enzyme activity (nanomole \[nmol\] 4-MU/hr) per mg of protein. On Day 1 of the first visit and at every visit thereafter, the samples were collected prior to dosing with migalastat. α-Gal A activity in leukocytes are presented by individual participants.

Participant 1: Baseline
GroupValue95% CI
Migalastat5.2
Participant 1: Week 12
GroupValue95% CI
Migalastat17.4
Participant 1: Week 96
GroupValue95% CI
Migalastat20.3
Participant 2: Baseline
GroupValue95% CI
Migalastat4.7
Participant 2: Week 12
GroupValue95% CI
Migalastat24.6
Participant 2: Week 96
GroupValue95% CI
Migalastat22.8
Participant 3: Baseline
GroupValue95% CI
Migalastat6.6
Participant 3: Week 12
GroupValue95% CI
Migalastat24.6

Adverse events — posted to ClinicalTrials.gov

Time frame: Day 1 after dosing through Week 96 (end of extension period). Reporting threshold: 5%. Adverse-event reports describe events observed during the trial — not all are caused by the drug.

Migalastat
Serious: 0/9 (0%)
Deaths:
Other adverse events (55 terms — click to expand)

ReactionSystemMigalastat
HeadacheNervous system disorders
NauseaGastrointestinal disorders
Abdominal pain upperGastrointestinal disorders
Back painMusculoskeletal and connective tissue disorders
DiarrhoeaGastrointestinal disorders
Dry mouthGastrointestinal disorders
InsomniaPsychiatric disorders
MyalgiaMusculoskeletal and connective tissue disorders
PainGeneral disorders
Pain in extremityMusculoskeletal and connective tissue disorders
VertigoEar and labyrinth disorders
AngiokeratomaSkin and subcutaneous tissue disorders
ArrhythmiaCardiac disorders
AsthmaRespiratory, thoracic and mediastinal disorders
Bipolar disorderPsychiatric disorders
Blood bilirubin increasedInvestigations
Blood creatine phosphokinase increasedInvestigations
Blood glucose increasedInvestigations
Chest wall painMusculoskeletal and connective tissue disorders
ConstipationGastrointestinal disorders
CoughRespiratory, thoracic and mediastinal disorders
DeafnessEar and labyrinth disorders
DyspepsiaGastrointestinal disorders
DysuriaRenal and urinary disorders
Ear painEar and labyrinth disorders
EpistaxisRespiratory, thoracic and mediastinal disorders
FatigueGeneral disorders
Femur fractureInjury, poisoning and procedural complications
GiardiasisInfections and infestations
Gingival painGastrointestinal disorders
Groin painMusculoskeletal and connective tissue disorders
HaematuriaRenal and urinary disorders
HungerGeneral disorders
HyperhidrosisSkin and subcutaneous tissue disorders
HypertensionVascular disorders
Influenza like illnessGeneral disorders
Libido decreasedPsychiatric disorders
Micturition urgencyRenal and urinary disorders
Muscle spasmsMusculoskeletal and connective tissue disorders
Musculoskeletal painMusculoskeletal and connective tissue disorders

Data from ClinicalTrials.gov NCT00214500 adverse events section.

Sponsor's own description

Study to evaluate the safety, tolerability, pharmacokinetics, and pharmacodynamics of migalastat hydrochloride (HCl) (migalastat) in participants with Fabry disease.

Publications & conference data

6 peer-reviewed publications reference this trial (live from Europe PMC):

  1. Treating lysosomal storage diseases with pharmacological chaperones: from concept to clinics.
    Parenti G. · · 2009 · cited 183× · PMID 20049730 · DOI 10.1002/emmm.200900036
  2. The validation of pharmacogenetics for the identification of Fabry patients to be treated with migalastat.
    Benjamin ER, Della Valle MC, Wu X, Katz E, et al · · 2017 · cited 164× · PMID 27657681 · DOI 10.1038/gim.2016.122
  3. Pharmacological enhancement of α-glucosidase by the allosteric chaperone N-acetylcysteine.
    Porto C, Ferrara MC, Meli M, Acampora E, et al · · 2012 · cited 84× · PMID 22990675 · DOI 10.1038/mt.2012.152
  4. A pharmacogenetic approach to identify mutant forms of α-galactosidase A that respond to a pharmacological chaperone for Fabry disease.
    Wu X, Katz E, Della Valle MC, Mascioli K, et al · · 2011 · cited 67× · PMID 21598360 · DOI 10.1002/humu.21530
  5. Digital pathology in nephrology clinical trials, research, and pathology practice.
    Barisoni L, Hodgin JB. · · 2017 · cited 27× · PMID 28858910 · DOI 10.1097/mnh.0000000000000360
  6. Abstracts of the 26th Annual North American Cystic Fibrosis Conference. October 11-13, 2012. Orlando, Florida, USA.
    · 2012 · PMID 23002459

Verify or expand the search:

Other trials of migalastat HCl

Trials testing the same drug.

Other recruiting trials for Fabry Disease

Currently open trials in the same condition.

Other Amicus Therapeutics trials

Trials by the same sponsor.

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Primary sources · FDA · ClinicalTrials.gov · EMA · SEC EDGAR · ChEMBL · Wikidata · full sourcing