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Cipaglucosidase Alfa

Phase 3

Pompe Disease (Late-onset) | Monoclonal antibody | Metabolic |Amicus Therapeutics, Inc.|Last Updated: Oct 27, 2025

Success Probability
Approval Probability 71%
TA Base Rate26%
Adjusted LOA41%
ML RiskLOW_RISK
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Market & Valuation
rNPV $3.2B
Market Size $9.4B
Revenue Basis $1.6B
Competitors 6
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Trial Design
RandomizedDouble-BlindACTIVE_CONTROLLEDDMCBiomarker
Total Trials2
Total Enrollment146
FDA Designations
No designations recorded
Clinical Trials (2)
NCT IDTitlePhaseStatusEnrollmentVelocityDesignStartCompletionLast UpdatedSitesCountries
NCT03911505ZIP Study-OL Study of Safety, PK, Efficacy, PD, Immunogenicity of ATB200/AT2221 in Pediatrics Aged 0 to < 18 y.o. w/LOPDPHASE3 ACTIVE NOT_RECRUITING 21Feb 13, 2020Jun 1, 2026Oct 27, 202517 United States, Australia +4
NCT03729362A Study Comparing ATB200/AT2221 With Alglucosidase Alfa/Placebo in Adult Subjects With Late-onset Pompe DiseasePHASE3 COMPLETED 125Dec 4, 2018Jan 15, 2021Sep 11, 202573 United States, Argentina +22
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Study Endpoints
Primary Endpoints
Incidence of treatment-emergent adverse events (TEAEs) from baseline
52 weeks
Change From Baseline to Week 52 in 6 Minute Walk Distance (6MWD)
Baseline, Week 52

The efficacy of cipaglucosidase alfa/miglustat co-administration on ambulatory function was measured by the 6MWT. The 6MWD, measured in meters, is the distance walked on the 6MWT. A greater distance indicated greater endurance. An increase from baseline indicated improvement.

Secondary Endpoints
Assessment of pharmacokinetic parameters
52 weeks
Change From Baseline to Week 52 in Sitting Forced Vital Capacity (FVC; % Predicted)
Baseline, Week 52
Change From Baseline to Week 52 in the Manual Muscle Test (MMT) Score for the Lower Extremities
Baseline, Week 52
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Study Design & Arms
AllocationNA
MaskingNONE
ModelSINGLE_GROUP
PurposeTREATMENT
Treatment Arms
ArmTypeDescription
Cipaglucosidase Alfa (ATB200)/Miglustat(AT2221)EXPERIMENTALParticipants received Cipaglucosidase Alfa (ATB200) co-administered with Miglustat (AT2221) capsule
Cipaglucosidase Alfa/MiglustatEXPERIMENTALParticipants received cipaglucosidase alfa co-administered with miglustat every 2 weeks (Q2W).
Alglucosidase Alfa/PlaceboACTIVE_COMPARATORParticipants received alglucosidase alfa co-administered with placebo Q2W.
Interventions
NameTypeDescription
Cipaglucosidase AlfaBIOLOGICALEnzyme Replacement Therapy via intravenous infusion
MiglustatDRUGParticipants received Cipaglucosidase Alfa (ATB200) co-administered with Miglustat(AT2221)
Alglucosidase AlfaBIOLOGICALParticipants received an IV infusion dose over a 4-hour duration Q2W.
PlaceboDRUGMiglustat matching placebo was administered orally 1 hour prior to alglucosidase alfa infusion Q2W.
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Eligibility Criteria
Age Range0 Years — 17 Years
SexALL
Healthy VolunteersNo
Study Sites17

Inclusion Criteria: 1. Male or female subjects (ERT-naïve \[have never received a dose of rhGAA\] or ERT-experienced \[have received rhGAA every 2 weeks for at least 6 months immediately before enrollment, and if ERT dosage has been modified, must have been on the modified dosage for at least 3 mon...

Countries:United StatesAustraliaCanadaGermanyItalyJapanArgentinaAustriaBelgiumBosnia and HerzegovinaBulgariaDenmarkFranceGreeceHungaryNetherlandsNew ZealandPolandSloveniaSouth KoreaSpainSwedenTaiwanUnited Kingdom
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Recent Changes (Last 90 Days)
LOWMay 26, 2026NCT03911505primaryCompletionDate: changed
LOWMay 24, 2026NCT03911505studyFirstPostDate: changed