NCT00283959

Brief Summary

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

Trial Health

90
On Track

Trial Health Score

Automated assessment based on enrollment pace, timeline, and geographic reach

Enrollment
4

participants targeted

Target at below P25 for phase_2

Timeline
Completed

Started Jun 2006

Geographic Reach
2 countries

2 active sites

Status
completed

Health score is calculated from publicly available data and should be used for screening purposes only.

Trial Relationships

Click on a node to explore related trials.

Study Timeline

Key milestones and dates

First Submitted

Initial submission to the registry

January 27, 2006

Completed
4 days until next milestone

First Posted

Study publicly available on registry

January 31, 2006

Completed
5 months until next milestone

Study Start

First participant enrolled

June 27, 2006

Completed
1.9 years until next milestone

Primary Completion

Last participant's last visit for primary outcome

May 8, 2008

Completed
Same day until next milestone

Study Completion

Last participant's last visit for all outcomes

May 8, 2008

Completed
10.3 years until next milestone

Results Posted

Study results publicly available

September 7, 2018

Completed
Last Updated

October 31, 2018

Status Verified

October 1, 2018

Enrollment Period

1.9 years

First QC Date

January 27, 2006

Results QC Date

August 10, 2018

Last Update Submit

October 1, 2018

Conditions

Keywords

Amicus TherapeuticsAT1001GalafoldMigalastatSubstrate

Outcome Measures

Primary Outcomes (1)

  • Number Of Participants Who Experienced Severe Treatment-emergent Adverse Events (TEAEs)

    TEAEs were defined as any adverse event with a 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 48 is presented. A summary of serious and all other non-serious adverse events regardless of causality is located in the Reported Adverse Events module.

    Day 1 (after dosing) through Week 48 (end of extension period)

Secondary Outcomes (1)

  • α-Galactosidase A (α-Gal A) Activity In Peripheral Blood Mononuclear Cells (PBMC) At Baseline, Week 12, And Week 48

    Baseline, Week 12 (end of treatment period), Week 48 (end of extension period)

Study Arms (1)

Migalastat

EXPERIMENTAL

Migalastat 150 mg was administered orally QOD during the 12-week treatment period and then during the optional 36-week extension period.

Drug: migalastat HCl

Interventions

Also known as: AT1001, Galafold, migalastat
Migalastat

Eligibility Criteria

Age18 Years - 65 Years
Sexmale
Healthy VolunteersNo
Age GroupsAdult (18-64), Older Adult (65+)

You may qualify if:

  • Males between 18 and 65 years of age (inclusive)
  • Hemizygous for Fabry disease
  • Had a confirmed diagnosis of Fabry disease with a documented missense gene mutation (individual or familial)
  • Had enhanceable enzyme activity based on in vitro tests
  • Had documented evidence of cardiac and/or renal dysfunction (for example, abnormal electrocardiogram (ECG), left ventricular hypertrophy, renal insufficiency) and/or cerebral tissue dysfunction documented by evidence of stroke and/or peripheral nervous tissue dysfunction (for example, intolerance to heat/cold, decrease of perspiration)
  • Must have been previously untreated by enzyme replacement therapy (ERT) or substrate depletion for Fabry disease or were able to stop ERT for at least 18 weeks or up to three months, and be willing to undergo two kidney and three skin biopsies
  • Agreed to be sexually abstinent or use a condom with spermicide when engaging in sexual activity during the course of the study and for a period of 30 days following their completion of the study
  • Were willing and able to sign an informed consent form

You may not qualify if:

  • History of significant disease other than Fabry disease
  • History of organ transplant
  • Serum creatinine \>2 mg per deciliter on Day -2
  • Screening 12-lead ECG demonstrating corrected QT interval \>450 milliseconds prior to dosing
  • Pacemaker or other contraindication for magnetic resonance imaging (MRI) scanning
  • Took any of the following prohibited medications: Fabrazyme® (agalsidase beta), Replagal™ (agalsidase alfa), Glyset® (miglitol), Zavesca® (miglustat), or any experimental therapy for any indication
  • Participated in a previous clinical trial in the last 30 days
  • Any other condition, which, in the opinion of the investigator, would jeopardize the safety of the participant or impact the validity of the study results

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (2)

Unknown Facility

Parkville, Australia

Location

Unknown Facility

Porto Alegre, Brazil

Location

Related Publications (2)

  • Benjamin ER, Della Valle MC, Wu X, Katz E, Pruthi F, Bond S, Bronfin B, Williams H, Yu J, Bichet DG, Germain DP, Giugliani R, Hughes D, Schiffmann R, Wilcox WR, Desnick RJ, Kirk J, Barth J, Barlow C, Valenzano KJ, Castelli J, Lockhart DJ. The validation of pharmacogenetics for the identification of Fabry patients to be treated with migalastat. Genet Med. 2017 Apr;19(4):430-438. doi: 10.1038/gim.2016.122. Epub 2016 Sep 22.

  • Germain DP, Giugliani R, Hughes DA, Mehta A, Nicholls K, Barisoni L, Jennette CJ, Bragat A, Castelli J, Sitaraman S, Lockhart DJ, Boudes PF. Safety and pharmacodynamic effects of a pharmacological chaperone on alpha-galactosidase A activity and globotriaosylceramide clearance in Fabry disease: report from two phase 2 clinical studies. Orphanet J Rare Dis. 2012 Nov 24;7:91. doi: 10.1186/1750-1172-7-91.

MeSH Terms

Conditions

Fabry Disease

Interventions

migalastatlarazotide acetate

Condition Hierarchy (Ancestors)

SphingolipidosesLysosomal Storage Diseases, Nervous SystemBrain Diseases, Metabolic, InbornBrain Diseases, MetabolicBrain DiseasesCentral Nervous System DiseasesNervous System DiseasesCerebral Small Vessel DiseasesCerebrovascular DisordersVascular DiseasesCardiovascular DiseasesGenetic Diseases, X-LinkedGenetic Diseases, InbornCongenital, Hereditary, and Neonatal Diseases and AbnormalitiesMetabolism, Inborn ErrorsLipidosesLipid Metabolism, Inborn ErrorsLysosomal Storage DiseasesMetabolic DiseasesNutritional and Metabolic DiseasesLipid Metabolism Disorders

Results Point of Contact

Title
Amicus Therapeutics
Organization
Medical Affairs

Study Officials

  • Medical Monitor, Clinical Research

    Amicus Therapeutics

    STUDY DIRECTOR

Publication Agreements

PI is Sponsor Employee
No
Restriction Type
OTHER
Restrictive Agreement
Yes

Study Design

Study Type
interventional
Phase
phase 2
Allocation
NA
Masking
NONE
Purpose
TREATMENT
Intervention Model
SINGLE GROUP
Sponsor Type
INDUSTRY
Responsible Party
SPONSOR

Study Record Dates

First Submitted

January 27, 2006

First Posted

January 31, 2006

Study Start

June 27, 2006

Primary Completion

May 8, 2008

Study Completion

May 8, 2008

Last Updated

October 31, 2018

Results First Posted

September 7, 2018

Record last verified: 2018-10

Locations