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UX016

Phase 1

GNE Myopathy | Small molecule | Other |Ultragenyx Pharmaceutical Inc.|Last Updated: Apr 6, 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_CONTROLLEDDMCBiomarker
Total Trials1
Total Enrollment24
FDA Designations
No designations recorded
Clinical Trials (1)
NCT IDTitlePhaseStatusEnrollmentVelocityDesignStartCompletionLast UpdatedSitesCountries
NCT07511556First-in-human Study of UX016 in GNEMPHASE1 NOT YET_RECRUITING 24Oct 1, 2026Dec 1, 2028Apr 6, 20262 United States
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Study Endpoints
Primary Endpoints
Number of Participants with Treatment-Emergent Adverse Events (TEAEs)
Up to Week 104
Upper Extremity Composite (UEC) Score Change From Baseline
Baseline, 48 Weeks
Secondary Endpoints
Free and Total Sialic Acid (SA) in Muscle (Quadriceps) Change From Baseline
Baseline, 12 Weeks
Lower Extremity Composite (LEC) Score Change From Baseline
Baseline, 48 Weeks
6-Minute Walk Test (6MWT) Change From Baseline
Baseline, 48 Weeks
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Study Design & Arms
AllocationRANDOMIZED
MaskingQUADRUPLE
ModelSEQUENTIAL
PurposeTREATMENT
Treatment Arms
ArmTypeDescription
Lower Dose UX016 -> Extension PeriodEXPERIMENTALParticipants will be randomized 3:1. Those randomized to lower dose will receive UX016 daily following up to two single dose administrations. After 48 weeks of daily dosing, participants will be eligible to enter the extension period.
Higher Dose UX016 -> Extension PeriodEXPERIMENTALParticipants will be randomized 3:1. Those randomized to higher dose will receive UX016 daily following a single dose administration. After 48 weeks of daily dosing, participants will be eligible to enter the extension period.
Placebo -> Extension PeriodPLACEBO_COMPARATORParticipants will be randomized 3:1. Those randomized to placebo will receive placebo per the same Lower or Higher Dose UX016 cohorts. After 48 weeks of daily dosing, participants will be eligible to enter the extension period and receive UX016 at the cohort assigned dose level.
Interventions
NameTypeDescription
UX016DRUGTablets for oral use
PlaceboOTHERTablets for oral use. Tablets will match the UX016 tablets, but contain no active ingredients
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Eligibility Criteria
Age Range18 Years — 55 Years
SexALL
Healthy VolunteersNo
Study Sites2

Inclusion Criteria: * A confirmed diagnosis of GNEM (also known as hereditary inclusion body myopathy \[HIBM\], distal myopathy with rimmed vacuoles \[DMRV\], inclusion body myopathy 2 \[IBM2\], and Nonaka myopathy in Japan) by Clinical Laboratory Improvement Amendments (CLIA)-certified genetic tes...

Countries:United States
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Competitive Landscape -Arrhythmogenic Right Ventricular Cardiomyopathy 3 trials
CompanyTickerTrialsLead PhaseDrugs
Lexeo Therapeutics, Inc.LXEO3PHASE1LX2020
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Recent Changes (Last 90 Days)
LOWMay 26, 2026NCT07511556primaryCompletionDate: changed
LOWMay 24, 2026NCT07511556studyFirstPostDate: changed