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zilganersen

Phase 3

Alexander Disease | Small molecule | Other |Ionis Pharmaceuticals, Inc.|Last Updated: Apr 3, 2026

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-BlindPLACEBO_CONTROLLEDBiomarker
Total Trials1
Total Enrollment54
FDA Designations
PRIORITY_REVIEW
Clinical Trials (1)
NCT IDTitlePhaseStatusEnrollmentVelocityDesignStartCompletionLast UpdatedSitesCountries
NCT04849741A Study to Evaluate the Safety and Efficacy of Zilganersen (ION373) in Patients With Alexander Disease (AxD)PHASE3 ACTIVE NOT_RECRUITING 54Jun 1, 2021Sep 1, 2029Apr 3, 202613 United States, Australia +6
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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 and long-term extension periods, where participants will receive zilganersen by ITB injection from Week 61 to Week 229.
PlaceboPLACEBO_COMPARATORMatching placebo will be administered by ITB injection once every 12 weeks through Week 49. It will be followed by the open-label and long-term extension periods, where participants will receive zilganersen by ITB injection from Week 61 to Week 229.
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 — 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)
LOWMay 26, 2026NCT04849741primaryCompletionDate: changed
LOWMay 24, 2026NCT04849741studyFirstPostDate: changed