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zilganersen

Phase 3

Alexander Disease | Small molecule | Rare Disease |Ionis Pharmaceuticals, Inc.|Last Updated: Aug 21, 2026

Success Probability

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Market & Valuation

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Trial Design

RandomizedDouble-BlindPLACEBO_CONTROLLED
Total Trials1
Total Enrollment54

FDA Designations

PRIORITY_REVIEWFAST_TRACKBREAKTHROUGH_THERAPY

Clinical trial landscape

zilganersen · 1 trial · 1 indication

Phase 3 1
NCT04849741A Study to Evaluate the Safety and Efficacy of Zilganersen (ION373) in Patients With Alexander Disease (AxD)Alexander Disease
ACTIVE NOT_RECRUITING54 Analytics
PHASE3ACTIVE NOT_RECRUITING
A Study to Evaluate the Safety and Efficacy of Zilganersen (ION373) in Patients With Alexander Disease (AxD)
Alexander DiseaseUnlock trial analytics

Study Endpoints

Primary Endpoints

Percent Change from Baseline in the 10-Meter Walk Test (10MWT)
Baseline and Week 61

10-Meter Walk Test (10MWT) is an assessment of gait speed.

Secondary Endpoints

Change From Baseline in Most Bothersome Symptom (MBS)
Baseline and Week 61
Change From Baseline in Patient Global Impression of Severity (PGIS) Score
Baseline and Week 61
Change From Baseline in Patient Global Impression of Change (PGIC) Score
Baseline and Week 61
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Study Design & Arms

AllocationRANDOMIZED
MaskingDOUBLE
ModelPARALLEL
PurposeTREATMENT

Treatment Arms

ArmTypeDescription
zilganersenEXPERIMENTALZilganersen will be administered by intrathecal bolus (ITB) injection once every 12 weeks through Week 49. The 60-week double-blind treatment period will be followed by the open-label, long-term and extended long-term extension periods, where participants will receive zilganersen by ITB injection from Week 61 to Week 469.
PlaceboPLACEBO_COMPARATORMatching placebo will be administered by ITB injection once every 12 weeks through Week 49. It will be followed by the open-label, long-term and extended long-term extension periods, where participants will receive zilganersen by ITB injection from Week 61 to Week 469.

Interventions

NameTypeDescription
zilganersenDRUGzilganersen will be administered by ITB injection.
PlaceboDRUGzilganersen-matching placebo will be administered by ITB injection.
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Eligibility Criteria

Age Range2 Years to 65 Years
SexALL
Healthy VolunteersNo
Study Sites13

Key Inclusion Criteria: 1. Clinical phenotype and brain imaging consistent with a diagnosis of Alexander disease 2. Documented genetic mutation in the GFAP gene 3. Aged ≥ 2 to 65 years old at the time of informed consent 4. Able and willing to meet all study requirements, including travel to Study ...

Countries:United StatesAustraliaCanadaIsraelItalyJapanNetherlandsUnited Kingdom
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Recent Changes (Last 90 Days)

MEDIUMAug 21, 2026NCT04849741Completion: 2029-09 → 2034-07
MEDIUMAug 21, 2026NCT04849741Completion: 2029-09 → 2034-07

Frequently asked questions about zilganersen

What is zilganersen used for?

Zilganersen is an investigational small molecule antisense oligonucleotide (ASO) being developed for the treatment of Alexander Disease, a rare neurological condition. It is currently in Phase 3 clinical development and has not been approved by the FDA.

How does zilganersen work?

Zilganersen is an antisense oligonucleotide (ASO), a class of small molecules that work by targeting specific genetic material to modulate protein production. Its mechanism is designed to address the underlying cause of Alexander Disease, though the specific molecular target is not disclosed.

Who is developing zilganersen?

Zilganersen is being developed by Ionis Pharmaceuticals, Inc. (NASDAQ: IONS). The company is conducting a Phase 3 clinical trial to evaluate the drug's safety and efficacy in patients with Alexander Disease.

What phase is zilganersen in?

Zilganersen is in Phase 3 clinical development. It is an investigational drug and has not been approved by the FDA. The drug has received Priority Review, Fast Track, and Breakthrough Therapy designations from the FDA.

What clinical trials is zilganersen in?

Zilganersen is being studied in a Phase 3 trial (NCT04849741), titled 'A Study to Evaluate the Safety and Efficacy of Zilganersen (ION373) in Patients With Alexander Disease (AxD)'. The trial is active but not recruiting, with an enrollment of 54 participants across multiple countries.

Is zilganersen the same as ION373?

Yes, zilganersen is also known as ION373. The Phase 3 clinical trial NCT04849741 evaluates both names, referring to the drug as 'Zilganersen (ION373)' in its title.