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aceneuramic acid

Phase 3

Hereditary Inclusion Body Myopathy | Small molecule | Rare Disease |Ultragenyx Pharmaceutical Inc.|Last Updated: Jun 27, 2019

Success Probability

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Trial Design

RandomizedDouble-BlindPLACEBO_CONTROLLEDDMC
Total Trials1
Total Enrollment89

FDA Designations

No designations recorded

Clinical trial landscape

aceneuramic acid · 1 trial · 4 indications

Phase 3 1
NCT02377921Phase 3 Randomized, Double-Blind, Placebo-Controlled Study to Evaluate Sialic Acid in Patients With Glucosamine (UDP-N-acetyl)-2-epimerase Myopathy (GNEM) or Hereditary Inclusion Body Myopathy (HIBM)Hereditary Inclusion Body Myopathy
COMPLETED89 Analytics
PHASE3COMPLETED
Phase 3 Randomized, Double-Blind, Placebo-Controlled Study to Evaluate Sialic Acid in Patients With Glucosamine (UDP-N-acetyl)-2-epimerase Myopathy (GNEM) or Hereditary Inclusion Body Myopathy (HIBM)
Hereditary Inclusion Body MyopathyUnlock trial analytics

Study Endpoints

Primary Endpoints

Change From Baseline in UEC Score (Total Force in kg) at Week 48
Baseline, Week 48

Muscle strength based on the maximum voluntary isometric contraction (MVIC) against a dynamometer was measured bilaterally in the following upper extremity muscle groups: gross grip, shoulder abductors, elbow flexors, and elbow extensors. The UEC is derived from the sum of the average of the right and left total force values (measured in kg).

Secondary Endpoints

Change From Baseline in Muscle Strength in the Knee Extensors at Week 48
Baseline, Week 48
Change From Baseline in LEC Score (Total Force in kg) at Week 48
Baseline, Week 48
Change From Baseline in GNEM FAS Mobility Domain Score at Week 48
Baseline, Week 48
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Study Design & Arms

AllocationRANDOMIZED
MaskingQUADRUPLE
ModelPARALLEL
PurposeTREATMENT

Treatment Arms

ArmTypeDescription
Aceneuramic Acid Extended-Release (Ace-ER)EXPERIMENTALAce-ER 6 g/day, divided 3 times per day (TID) for 48 weeks.
PlaceboPLACEBO_COMPARATORMatching placebo TID for 48 weeks.

Interventions

NameTypeDescription
aceneuramic acid extended-release (Ace-ER)DRUGtablets for oral use
PlaceboDRUGtablets for oral use
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Eligibility Criteria

Age Range18 Years to 55 Years
SexALL
Healthy VolunteersNo
Study Sites13

Inclusion Criteria: * Male or female, aged 18 to 55 years, inclusive * Willing and able to provide written, signed informed consent after the nature of the study has been explained, and before any research-related procedures are conducted * Have a documented diagnosis of GNEM, HIBM, distal myopathy...

Countries:United StatesBulgariaCanadaFranceIsraelItalyUnited Kingdom
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Frequently asked questions about aceneuramic acid

What is aceneuramic acid used for?

Aceneuramic acid is an investigational small molecule being studied for the treatment of Hereditary Inclusion Body Myopathy, also known as GNE Myopathy. It is being developed by Ultragenyx Pharmaceutical Inc. (NASDAQ: RARE) for this rare disease indication.

What does aceneuramic acid target?

Aceneuramic acid is a form of sialic acid, a sugar molecule that is deficient in patients with Hereditary Inclusion Body Myopathy. The drug is designed to replace this missing sialic acid, addressing the underlying metabolic defect in the condition.

Who makes aceneuramic acid?

Aceneuramic acid is being developed by Ultragenyx Pharmaceutical Inc., a biopharmaceutical company traded on NASDAQ under the ticker RARE. The company focuses on developing therapies for rare and ultra-rare diseases.

What phase is aceneuramic acid in?

Aceneuramic acid is in Phase 3 clinical development. It has completed one Phase 3 trial, but it is still considered investigational and has not been approved by regulatory authorities. The drug is not yet available for commercial use.

What clinical trials is aceneuramic acid in?

Aceneuramic acid has been studied in one completed Phase 3 clinical trial, registered as NCT02377921. This was a randomized, double-blind, placebo-controlled study that enrolled 89 patients with Hereditary Inclusion Body Myopathy across multiple countries, including the United States, Canada, and European nations.

Is aceneuramic acid the same as sialic acid?

Aceneuramic acid is a form of sialic acid, specifically the free acid form of N-acetylneuraminic acid. In the context of Hereditary Inclusion Body Myopathy, it is used to supplement deficient sialic acid levels in patients.