The primary objectives of this study are to evaluate the safety of single IV doses of UX701 in patients with Wilson disease, to select the UX701 dose with the best benefit/risk profile based on the totality of safety and efficacy data and to evaluate the effect of UX701 on copper regulation.
Stage 1 (Phase 1/2) is an open-label safety and dose-finding stage designed to evaluate the safety and efficacy of 4 dose levels of UX701 to establish initial safety of UX701 and select a safe and efficacious dose for further evaluation. Stage 2 (Phase 3) is a randomized, open-label, active-controlled stage to evaluate the safety and efficacy of UX701 using the dose selected in Stage 1. Stage 3 is a long-term follow-up stage designed to evaluate the safety, efficacy, and clinical benefit of UX701 for at least 5 years from the time of UX701 administration. Participants who receive UX701 will receive premedication, prophylactic oral corticosteroids and immunomodulation therapy.
Study Type
INTERVENTIONAL
Allocation
RANDOMIZED
Purpose
TREATMENT
Masking
SINGLE
Enrollment
82
Nonreplicating, recombinant gene transfer vector
SOC treatment (i.e., copper chelators and/or zinc) administered according to standard regimens.
University of California Los Angeles
Los Angeles, California, United States
Stanford University
Redwood City, California, United States
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
Time frame: Up to Week 52
Stage 1: Change in 24-hour Urinary Copper Concentration from Baseline at Week 52
Time frame: Baseline, Week 52
Stage 1: Change in Total Copper from Baseline at Week 52
Time frame: Baseline, Week 52
Stage 1: Change in Ceruloplasmin-bound Copper from Baseline at Week 52
Time frame: Baseline, Week 52
Stage 1: Change in Ceruloplasmin from Baseline at Week 52
Time frame: Baseline, Week 52
Stage 1: Change in Non-Ceruloplasmin-bound Copper (NCC) from Baseline at Week 52
Time frame: Baseline, Week 52
Stage 1: Change in Free Copper from Baseline at Week 52
Time frame: Baseline, Week 52
Stage 1: Change in Ceruloplasmin Activity from Baseline at Week 52
Time frame: Baseline, Week 52
Stage 1: Percent Reduction in Standard of Care (SOC) Medication by Week 52
Time frame: Week 52
Stage 1: Number of Participants Who Discontinue SOC Medication by Week 52
Time frame: Week 52
Stage 1: Number of Consecutive Weeks off SOC Medication at Week 52
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University of California Davis
Sacramento, California, United States
Northwestern University
Chicago, Illinois, United States
Indiana University
Indianapolis, Indiana, United States
Massachusetts General Hospital
Boston, Massachusetts, United States
University of Michigan
Ann Arbor, Michigan, United States
Duke University Medical Center
Durham, North Carolina, United States
Vanderbilt University Medical Center
Nashville, Tennessee, United States
University of Utah
Salt Lake City, Utah, United States
...and 6 more locations
Time frame: Week 52
Stage 2: Change in 24-hour Urinary Copper Concentration from Baseline at Week 52, Evaluated for Superiority
Time frame: Baseline, Week 52
Stage 2: Percent Reduction in SOC Medication by Week 52, Evaluated for Superiority
Time frame: Week 52
Stage 2: Change in Ceruloplasmin Activity Levels from Baseline at Week 52, Evaluated for Superiority
Time frame: Baseline, Week 52
Stage 2: Number of Participants who Discontinue SOC Medication by Week 52
Time frame: Week 52
Stage 2: Change in FACIT-Fatigue Scale Score from Baseline at Week 52
Time frame: Baseline, Week 52
Stage 2: Change in Liver Copper Concentration Assessed by Liver Biopsy from Baseline at Week 52
Time frame: Baseline, Week 52