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UX701

Phase 1

Wilson Disease | Gene therapy | Rare Disease |Ultragenyx Pharmaceutical Inc.|Last Updated: Jun 15, 2026

Success Probability

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Market & Valuation

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Trial Design

RandomizedCONTROLLEDDMC
Total Trials1
Total Enrollment82

FDA Designations

No designations recorded

Clinical trial landscape

UX701 · 1 trial · 1 indication

Phase 1 1
NCT04884815A Phase 1/2/3 Study of UX701 Gene Therapy in Adults With Wilson DiseaseWilson Disease
ACTIVE NOT_RECRUITING82 Analytics
PHASE1ACTIVE NOT_RECRUITING
A Phase 1/2/3 Study of UX701 Gene Therapy in Adults With Wilson Disease
Wilson DiseaseUnlock trial analytics

Study Endpoints

Primary Endpoints

Stage 1: Incidence of Treatment-Emergent Adverse Events (TEAEs), Treatment-Emergent Serious Adverse Events (TESAEs), Adverse Events of Special Interest (AESIs), Treatment-Related TEAEs, and Treatment-Related TESAEs
Up to Week 52
Stage 1: Change in 24-hour Urinary Copper Concentration from Baseline at Week 52
Baseline, Week 52
Stage 1: Change in Total Copper from Baseline at Week 52
Baseline, Week 52
Stage 1: Change in Ceruloplasmin-bound Copper from Baseline at Week 52
Baseline, Week 52
Stage 1: Change in Ceruloplasmin from Baseline at Week 52
Baseline, Week 52
Stage 1: Change in Non-Ceruloplasmin-bound Copper (NCC) from Baseline at Week 52
Baseline, Week 52
Stage 1: Change in Free Copper from Baseline at Week 52
Baseline, Week 52
Stage 1: Change in Ceruloplasmin Activity from Baseline at Week 52
Baseline, Week 52
Stage 1: Percent Reduction in Standard of Care (SOC) Medication by Week 52
Week 52
Stage 1: Number of Participants Who Discontinue SOC Medication by Week 52
Week 52
Stage 1: Number of Consecutive Weeks off SOC Medication at Week 52
Week 52
Stage 2: Change in 24-hour Urinary Copper Concentration from Baseline at Week 52, Evaluated for Superiority
Baseline, Week 52
Stage 2: Percent Reduction in SOC Medication by Week 52, Evaluated for Superiority
Week 52

Secondary Endpoints

Stage 2: Change in Ceruloplasmin Activity Levels from Baseline at Week 52, Evaluated for Superiority
Baseline, Week 52
Stage 2: Number of Participants who Discontinue SOC Medication by Week 52
Week 52
Stage 2: Change in FACIT-Fatigue Scale Score from Baseline at Week 52
Baseline, Week 52
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Study Design & Arms

AllocationRANDOMIZED
MaskingSINGLE
ModelSEQUENTIAL
PurposeTREATMENT

Treatment Arms

ArmTypeDescription
Stage 1: UX701 Dose Level 1EXPERIMENTALParticipants receive a single, peripheral intravenous (IV) infusion of UX701 at dose level 1.
Stage 1: UX701 Dose Level 2EXPERIMENTALParticipants receive a single, peripheral IV infusion of UX701 at dose level 2.
Stage 1: UX701 Dose Level 3EXPERIMENTALParticipants receive a single, peripheral IV infusion of UX701 at dose level 3.
Stage 1: UX701 Dose Level 4EXPERIMENTALParticipants receive a single, peripheral IV infusion of UX701 at dose level 4.
Stage 2: UX701 at Selected DoseEXPERIMENTALParticipants randomized to UX701 receive a single, peripheral IV infusion of UX701 at the selected dose.
Stage 2: Standard of Care (SOC) to UX701EXPERIMENTALParticipants randomized to SOC will continue their baseline SOC medications for 52 weeks, followed by a single, peripheral IV infusion of UX701 at the selected dose. Following UX701 administration, participants will be evaluated for modification of their SOC medications.

Interventions

NameTypeDescription
UX701GENETICNonreplicating, recombinant gene transfer vector
Standard of Care (SOC)DRUGSOC treatment (i.e., copper chelators and/or zinc) administered according to standard regimens.
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Eligibility Criteria

Age Range18 Years to N/A
SexALL
Healthy VolunteersNo
Study Sites16

Key Inclusion Criteria: * Confirmed diagnosis of Wilson disease based on genetic confirmation of heterozygous or homozygous biallelic ATP7B mutation. * Stable Wilson disease as evidenced by ongoing copper chelator (ie, penicillamine, trientine) and/or zinc therapy for at least 2 months at screening...

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

LOWJun 16, 2026NCT04884815lastUpdatePostDate: changed
LOWJun 16, 2026NCT04884815lastUpdatePostDate: changed
LOWJun 16, 2026NCT04884815lastUpdatePostDate: changed
LOWJun 16, 2026NCT04884815lastUpdatePostDate: changed

Frequently asked questions about UX701

What is UX701 used for?

UX701 is an investigational gene therapy being developed for the treatment of Wilson Disease, a rare inherited disorder that causes copper to accumulate in the body. It is designed to address the underlying genetic cause of the condition. The therapy is currently in clinical development and has not been approved by regulatory authorities.

How does UX701 work?

UX701 is a gene therapy intended to deliver a functional copy of the gene that is defective in Wilson Disease. By providing this gene, the therapy aims to restore the body's ability to properly process and eliminate copper, potentially addressing the root cause of the disease. This mechanism is based on the therapy's design as a gene replacement approach.

Who is developing UX701?

UX701 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 clinical trials to evaluate the safety and efficacy of UX701 in patients with Wilson Disease.

What phase is UX701 in?

UX701 is currently in Phase 1 of clinical development. The ongoing trial is a Phase 1/2/3 study, meaning it is designed to evaluate the therapy across multiple phases in a single trial. The study is active but not recruiting participants, and the therapy remains investigational.

What clinical trials is UX701 in?

UX701 is being studied in a Phase 1/2/3 clinical trial with the identifier NCT04884815. This trial is enrolling adults with Wilson Disease and is being conducted in the United States, Portugal, Spain, and the United Kingdom. The study has a planned enrollment of 82 participants and is currently active but not recruiting.

Is UX701 the same as other gene therapies for Wilson Disease?

UX701 is a specific gene therapy developed by Ultragenyx for Wilson Disease. It is not known to be the same as other gene therapies for this condition. The therapy is being evaluated in its own clinical trial and has a distinct mechanism of delivering a functional gene to address the disease.