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UX016

Phase 1

GNE Myopathy | Small molecule | Rare Disease |Ultragenyx Pharmaceutical Inc.|Last Updated: Sep 3, 2026

Success Probability

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Market & Valuation

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Trial Design

RandomizedDouble-BlindPLACEBO_CONTROLLEDDMC
Total Trials1
Total Enrollment24

FDA Designations

No designations recorded

Clinical trial landscape

UX016 · 1 trial · 1 indication

Phase 1 1
NCT07511556First-in-human Study of UX016 in GNEMGNE Myopathy
RECRUITING24 Analytics
PHASE1RECRUITING
First-in-human Study of UX016 in GNEM
GNE MyopathyUnlock trial analytics

Study Endpoints

Primary Endpoints

Number of Participants with Treatment-Emergent Adverse Events (TEAEs)
Up to Week 96 (Double Blind and Extension Periods)
Upper Extremity Composite (UEC) Score Change From Baseline
Baseline, 48 Weeks

Secondary Endpoints

Plasma Area Under the Curve (AUC) of UX016 and Free Sialic Acid (SA)
Baseline, 12 Weeks
Plasma Maximum Concentration (Cmax) of UX016 and SA
Baseline, 12 Weeks
Excretion in Urine of UX016 and SA
Baseline, 12 Weeks
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Study Design & Arms

AllocationRANDOMIZED
MaskingQUADRUPLE
ModelSEQUENTIAL
PurposeTREATMENT

Treatment Arms

ArmTypeDescription
1g UX016 -> Extension PeriodEXPERIMENTALParticipants will be randomized 3:1 to receive UX016 or placebo. Depending on sub-cohort assignment, participants may receive two single doses of UX016 (0.5g and 1g) or one single dose (1g) before starting daily dosing, or they may proceed directly to daily dosing at the assigned 1g dose level. After completing 48 weeks of daily dosing, participants will be eligible to enter the Extension Period, during which all participants will receive UX016.
2g UX016 -> Extension PeriodEXPERIMENTALParticipants will be randomized 3:1 to receive UX016 or placebo. Depending on sub-cohort assignment, participants may receive a single dose of UX016 (2g) before starting daily dosing, or they may proceed directly to daily dosing at the assigned 2g dose level. After 48 weeks of daily dosing, participants will be eligible to enter the Extension Period, during which all participants will receive UX016.
Placebo -> Extension PeriodPLACEBO_COMPARATORParticipants will be randomized 3:1 to receive UX016 or placebo. Depending on sub-cohort assignment, participants may receive one or more single doses of placebo before starting daily dosing or they may proceed directly to daily dosing of placebo at the assigned dose level. After 48 weeks of daily dosing of placebo, participants will be eligible to enter the Extension Period, during which all participants will receive UX016.

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 to 55 Years
SexALL
Healthy VolunteersNo
Study Sites2

Inclusion Criteria: * A confirmed diagnosis of GNEM (also known as distal myopathy with rimmed vacuoles \[DMRV\], hereditary inclusion body myopathy \[HIBM\], 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 (matched to "GNE Myopathy")

CompanyTickerTrialsLead PhaseDrugs
Lexeo Therapeutics, Inc.LXEO3PHASE1LX2020
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Recent Changes (Last 90 Days)

LOWSep 3, 2026NCT07511556Status: NOT_YET_RECRUITING → RECRUITING
LOWSep 3, 2026NCT07511556Status: NOT_YET_RECRUITING → RECRUITING

Frequently asked questions about UX016

What is UX016 used for in GNE Myopathy?

UX016 is an investigational small molecule being developed for the treatment of GNE Myopathy, a rare inherited muscle-wasting disorder. It is currently in Phase 1 clinical development and has not been approved by regulatory authorities. The drug is being studied in a first-in-human trial to evaluate its safety and tolerability in patients.

What does UX016 target?

The specific molecular target of UX016 has not been disclosed. It is a small molecule therapeutic being developed by Ultragenyx Pharmaceutical Inc. for GNE Myopathy, a rare genetic condition. The drug's mechanism of action is not publicly detailed, and it is currently being evaluated in early-stage clinical trials.

Who makes UX016?

UX016 is being developed by Ultragenyx Pharmaceutical Inc., a biopharmaceutical company focused on rare diseases. The company's stock is traded under the ticker symbol RARE. Ultragenyx is conducting a Phase 1 clinical trial of UX016 in patients with GNE Myopathy in the United States.

What phase is UX016 in?

UX016 is in Phase 1 clinical development. It is an investigational drug and has not been approved by the FDA or any other regulatory body. The ongoing Phase 1 trial is a first-in-human study designed to assess the safety, tolerability, and pharmacokinetics of UX016 in patients with GNE Myopathy.

What clinical trials is UX016 in?

UX016 is being studied in one active Phase 1 clinical trial with the identifier NCT07511556. This first-in-human study is recruiting 24 participants with GNE Myopathy in the United States. The trial is randomized, double-blind, and placebo-controlled, enrolling adults aged 18 years and older.

Is UX016 the same as any other drug?

No alternative names for UX016 have been reported. It is a distinct investigational small molecule being developed by Ultragenyx Pharmaceutical Inc. for GNE Myopathy. As it is still in early clinical development, it is not yet marketed under any brand name.