Metabolic

Pompe Disease

Also known as Glycogen storage disease type II, Acid maltase deficiency, GSD II

Pompe disease is caused by mutations in the GAA gene that encodes the enzyme acid alpha-glucosidase. Without it, glycogen accumulates in lysosomes, progressively destroying muscle cells.

ORPHA:365 ↗Gene GAAPrevalence 1-9 per 100,000 (Orphanet)Onset Neonatal to Adult (variable)Genetic (autosomal recessive)

0

studies recruiting now

as of 7 Sept 2026

2

studies registered in total

as of 7 Sept 2026

0

countries with a recruiting site

as of 7 Sept 2026

None

recruiting study posted to date

among recruiting studies

Recruiting trials

No recruiting trial found right now.

2 studies are registered for Pompe Disease, but none was recruiting as of 7 Sept 2026. Here is what is still worth doing.

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Support

Patient organisations

Acid Maltase Deficiency AssociationPatient association
Visit website ↗

Registry: Pompe Registry (Sanofi Genzyme) · Join ↗. Registries connect patients to researchers and often hear about trials first.

About Pompe Disease

Pompe disease is caused by mutations in the GAA gene that encodes the enzyme acid alpha-glucosidase. Without it, glycogen accumulates in lysosomes, progressively destroying muscle cells. Infantile-onset Pompe presents within months of birth with severe cardiac and muscle weakness. Late-onset disease appears in childhood through adulthood with progressive limb and respiratory muscle weakness.

Common clinical features

Progressive proximal muscle weaknessMuscle weaknessExercise intoleranceDifficulty climbing stairsLower limb muscle weaknessDecreased circulating acid maltase activityOligosacchariduriaRespiratory insufficiency

From Orphanet’s phenotype annotations (CC BY 4.0). Not a complete list.

Treatments being studied

3 approved treatments and 16 in clinical development, from Open Targets (CC BY 4.0). Not medical advice.

Approved: Cipaglucosidase Alfa (Pombiliti)Approved: Alglucosidase Alfa (Myozyme)Approved: Avalglucosidase Alfa (Nexviadyme)
Phase 3Miglustat (Miglustat dipharma)
Phase 3Duvoglustat
Phase 3Reveglucosidase Alfa
Phase 2Clenbuterol (Spiropent)
Phase 2Zocaglusagene Nuzaparvovec
Phase 2Duvoglustat Hydrochloride
Phase 1/2Vanglusagene Ensiparvovec
Phase 1/2Albuterol (Aerolin)

+ 8 more in development

Before you apply

Things trial teams commonly ask about for Pompe Disease. Not eligibility rules; those are set by each study.

  • Infantile-onset versus late-onset Pompe are treated as distinct conditions in most trials
  • Prior ERT treatment and antibody status significantly affect trial eligibility
  • Respiratory function tests (FVC%) are a key eligibility and outcome measure

Related conditions

Information, not medical advice. Trial listings are shown as recorded on ClinicalTrials.gov; whether any study is right for you is a decision for you and your clinicians, and eligibility is decided by each research team. Disease information from Orphanet (CC BY 4.0).