TheraRadar
Data updated: May 26, 2026

OPFOLDA (miglustat)

Glucosylceramide Synthase Inhibitors
Rare Disease Approved 2023-09-28

Opfolda is an enzyme stabilizer used for adults with late-onset Pompe disease who weigh at least 40 kg. It helps patients with this specific enzyme deficiency when their current enzyme replacement therapy is no longer providing sufficient improvement. This medication is taken in combination with Pombiliti to help manage the condition more effectively.

Source: FDA Label • AMICUS THERAP US • Glucosylceramide Synthase Inhibitor

How OPFOLDA Works

This drug works by binding to and stabilizing the enzyme cipaglucosidase alfa-atga in the bloodstream, which prevents it from becoming inactive after infusion. Once the enzyme is transported into the lysosomes, the miglustat separates from it, allowing the enzyme to perform its function. While miglustat itself does not break down glycogen, it ensures the accompanying enzyme therapy remains stable enough to reach its target.

1
Indication
--
Phase 3 Trials
2
Years on Market

Details

Status
Prescription
First Approved
2023-09-28
Patent Cliff
2038

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Routes
ORAL
Dosage Forms
CAPSULE

Companies

Active Ingredient: MIGLUSTAT

OPFOLDA Approval History

2024
2025
2026
Original
New Indication
New Form
Label Update
2 FDA actions from 2023 to 2024
Jul 2024 SUPPL
Label · Labeling
Sep 2023 ORIGINAL
Update · Type 5 - New Formulation or New Manufacturer

What OPFOLDA Treats

1 indications

OPFOLDA is approved for 1 conditions since its original approval in 2023. These indications span multiple therapeutic areas including oncology, immunology, and more.

  • Pompe Disease
Source: FDA Label

OPFOLDA Competitive Set

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Indication competitors

4

Same indication, different mechanism — what else might this patient receive?

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Filters applied: drops same-active-ingredient (505(b)(2) reformulations), route-mismatch (topical vs systemic), and cross-therapeutic-area matches in same-indication rings.

Drugs Similar to OPFOLDA

3 of 4

FDA-approved drugs for similar conditions. Compare mechanisms and indications to understand treatment alternatives.

LUMIZYME
ALGLUCOSIDASE ALFA
1 shared
Sanofi
Shared indications:
Pompe Disease
MYOZYME
ALGLUCOSIDASE ALFA
1 shared
Sanofi
Shared indications:
Pompe Disease
NEXVIAZYME
AVALGLUCOSIDASE ALFA-NGPT
1 shared
Sanofi
Shared indications:
Pompe Disease
📋

Clinical Trial Registry

9 trials
Trial Sponsor ID Phase Status Title
NCT04808505 ROSSELLA ATB200-08 Ph 3 recruiting A Study to Evaluate the Safety, Efficacy, PK, PD and Immunogenicity of Cipaglucosidase Alfa/Miglustat in IOPD Subjects Aged 0 to <18
NCT03911505 ATB200-04 Ph 3 active not recruiting ZIP Study-OL Study of Safety, PK, Efficacy, PD, Immunogenicity of ATB200/AT2221 in Pediatrics Aged 0 to < 18 y.o. w/LOPD
NCT03822013 EMTISTD 97-01-30-36953 Ph 3 terminated Effects of Miglustat Therapy on Infantile Type of Sandhoff and Taysachs Diseases (EMTISTD)
NCT03729362 PROPEL results posted ATB200-03 Ph 3 completed A Study Comparing ATB200/AT2221 With Alglucosidase Alfa/Placebo in Adult Subjects With Late-onset Pompe Disease
NCT03910621 AC-056C405 Ph 4 completed Safety and Efficacy of Miglustat in Chinese NPC Patients
NCT02030015 Syner-G results posted Syner_G_Regimen U54NS065768, 1311M46101 Ph 4 terminated Synergistic Enteral Regimen for Treatment of the Gangliosidoses
NCT00742092 AC-056A202 Ph 2 completed Miglustat in Cystic Fibrosis
NCT01760564 200802043M Ph 3 completed Application of Miglustat in Patients With Niemann-Pick Type C
NCT00945347 MIG-99 Ph 2 completed Does a Nasal Instillation of Miglustat Normalize the Nasal Potential Difference in Cystic Fibrosis Patients ?
🔬

Active Pipeline

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Key Completed Trials

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Trial Timeline

Full development history with FDA approval milestones

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Understanding FDA Approval Types
Count Type What it means
- ORIG Original approval - drug first enters market
- SUPPL - Efficacy New indication (new disease/condition approved)
- SUPPL - Labeling Label text changes (warnings, dosing updates)
- SUPPL - Manufacturing Production changes (new facility)
- SUPPL - Chemistry Formulation changes (new dosage strength)

Green lines in the timeline show ORIG and Efficacy approvals - the clinically meaningful milestones.

OPFOLDA FDA Label Details

Indications & Usage

FDA Label (PDF)

OPFOLDA is indicated for the treatment of Pompe Disease.

View full patent landscape →
10 OB patents · 4 families · 237 international docs across 42 countries

OPFOLDA Patents & Exclusivity

Latest Patent: Sep 2038
Exclusivity: Sep 2026

Patents (10 active)

US12246062 Expires Sep 16, 2038
US10857212 Expires Aug 12, 2037
US12414985 Expires Dec 29, 2036
US11278601 Expires Dec 29, 2036
US11753632 Expires Sep 30, 2035
US10961522 Expires Sep 30, 2035
US10208299 Expires Sep 30, 2035
US12419937 Expires Mar 7, 2033
US11278599 Expires Mar 7, 2033
US10512677 Expires Mar 7, 2033

Exclusivity

NP Until Sep 2026
Source: FDA Orange Book

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Patent Timeline

  • Cliff: 2038
  • 10 active patents

Trial Analysis

  • Clinical trial tracking
  • Development stage analysis

Competitive Landscape

  • 4 similar drugs
  • Same target/indication analysis
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Data Sources

Data sourced from official FDA and NIH databases. Click links to verify on original sources.