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rAAV2/5-hNAGLU

Phase 1

Sanfilippo Syndrome B | Small molecule | Other |uniQure N.V.|Last Updated: Dec 2, 2019

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
UNCONTROLLEDDMCBiomarker
Total Trials1
Total Enrollment4
FDA Designations
No designations recorded
Clinical Trials (1)
NCT IDTitlePhaseStatusEnrollmentVelocityDesignStartCompletionLast UpdatedSitesCountries
NCT03300453Intracerebral Gene Therapy in Children With Sanfilippo Type B SyndromePHASE1 COMPLETED 4Sep 17, 2013Nov 27, 2019Dec 2, 20191 France
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Study Endpoints
Primary Endpoints
Number of Participants With Treatment-Related (Serious) Adverse Events as assessed by continuous evaluation of change from baseline
Baseline until end of study (Month 66)

Multiple measurements will be aggregated to derive the number of participants with Abnormal Laboratory Values and/or Adverse Events that are related to Treatment.

Secondary Endpoints
Number of Participants with presence of brain atrophy, white matter lesions and other lesions as assessed by cerebral MRI
Baseline until end of study (Month 66)
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Study Design & Arms
AllocationNA
MaskingNONE
ModelSINGLE_GROUP
PurposeTREATMENT
Treatment Arms
ArmTypeDescription
rAAV2/5-hNAGLUEXPERIMENTALEach patient will receive 960 µL of vector suspension. The vector suspension will be deposited simultaneously at 16 sites, each deposit containing 2.4x 1011 vg (4x1012 vg in total).
Interventions
NameTypeDescription
rAAV2/5-hNAGLUDRUGone-time brain intraparenchymal gene therapy dose
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Eligibility Criteria
Age Range18 Months — 60 Months
SexALL
Healthy VolunteersNo
Study Sites1

Inclusion criteria: * Age: 18 months up to 60 months (5th birthday); * Onset of clinical manifestations related to mucopolysaccharidosis type IIIB (MPSIIIB); * NAGLU activity in peripheral blood cell and/or cultured fibroblast extracts of less than 10% of controls; * Patient affiliated to, or cover...

Countries:France
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