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UX111

Phase 2

MPS IIIA | Monoclonal antibody | Rare Disease |Ultragenyx Pharmaceutical Inc.|Last Updated: Apr 24, 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
CONTROLLEDDMCBiomarker
Total Trials1
Total Enrollment36
FDA Designations
RMATFAST_TRACKORPHAN_DRUGRARE_PEDIATRIC_DISEASEACCELERATED_APPROVAL
Clinical Trials (1)
NCT IDTitlePhaseStatusEnrollmentVelocityDesignStartCompletionLast UpdatedSitesCountries
NCT02716246Phase I/II/III Gene Transfer Clinical Trial of scAAV9.U1a.hSGSHPHASE2 RECRUITING 36Apr 25, 2016Jul 1, 2027Apr 24, 20265 United States, Australia +1
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Study Endpoints
Primary Endpoints
Cerebrospinal Fluid (CSF) Heparan Sulfate (HS) (Disaccharide) Exposure
Up to Month 24 Visit

Exposure is defined as the time-normalized area under the curve (AUC) of the percentage reduction from baseline.

Secondary Endpoints
Bayley Scales of Infant and Toddler Development-Third Edition (BSITD-III) Cognitive Raw Score Over Time
Up to Month 24 Visit
CSF Ganglioside Type 2 (GM2) Exposure
Up to Month 24 Visit
CSF Ganglioside Type 3 (GM3) Exposure
Up to Month 24 Visit
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Study Design & Arms
AllocationNON_RANDOMIZED
MaskingNONE
ModelSEQUENTIAL
PurposeTREATMENT
Treatment Arms
ArmTypeDescription
Cohort 1 Low DoseEXPERIMENTALDose of 0.5 X 10\^13 vg/kg
Cohort 2 Mid DoseEXPERIMENTALDose of 1 X 10\^13 vg/kg
Cohort 3 High DoseEXPERIMENTALDose of 3 X 10\^13 vg/kg
Cohort 4 High Dose (Spain Only)EXPERIMENTALDose of 3 X 10\^13 vg/kg
Interventions
NameTypeDescription
UX111BIOLOGICALSelf-complementary adeno-associated virus serotype 9 carrying the human SGSH gene under the control of a U1a promoter (scAAV9.U1a.hSGSH) will be delivered one time through a venous catheter inserted into a peripheral limb vein.
Prophylactic Immunomodulatory (IM) TherapyDRUG -
Optimized Prophylactic IM TherapyDRUG -
Adjuvant IM TherapyDRUGThe Principal Investigator and/or caregiver, in consultation with the medical monitor, will determine whether to initiate adjuvant IM therapy. Not all participants may receive adjuvant IM therapy.
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Eligibility Criteria
SexALL
Healthy VolunteersNo
Study Sites5

Inclusion Criteria: * Diagnosis of MPS IIIA confirmed by the following methods: * No detectable or significantly reduced SGSH enzyme activity by leukocyte assay, and * Genomic DNA analysis demonstrating homozygous or compound heterozygous mutations in the SGSH gene (based upon review of docume...

Countries:United StatesAustraliaSpain
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Recent Changes (Last 90 Days)
LOWMay 26, 2026NCT02716246primaryCompletionDate: changed
LOWMay 24, 2026NCT02716246studyFirstPostDate: changed