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AVXS-101

Phase 1

Spinal Muscular Atrophy 1 | Monoclonal antibody | Neurology |Novartis AG|Last Updated: Sep 15, 2022

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 Enrollment15
FDA Designations
No designations recorded
Clinical Trials (1)
NCT IDTitlePhaseStatusEnrollmentVelocityDesignStartCompletionLast UpdatedSitesCountries
NCT02122952Gene Transfer Clinical Trial for Spinal Muscular Atrophy Type 1PHASE1 COMPLETED 15May 5, 2014Dec 15, 2017Sep 15, 20221 United States
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Study Endpoints
Primary Endpoints
Number of Participants That Experienced One Grade III or Higher Unanticipated, Treatment-related Toxicity That Presents With Clinical Symptoms and Requires Medical Treatment
2 years
Secondary Endpoints
Number of Participants Who Experienced Permanent Ventilation or Death
Up to 13.6 months of age
Percent Change From Baseline in Mean Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP-INTEND) Score
Baseline to 24 months post-dose
Number of Participants With Assessed Improvement in Motor Function
24 months post-dose
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Study Design & Arms
AllocationNON_RANDOMIZED
MaskingNONE
ModelSINGLE_GROUP
PurposeTREATMENT
Treatment Arms
ArmTypeDescription
Cohort 1EXPERIMENTAL6.7 X 10\^13 vg/kg of AVXS-101 delivered one-time through a venous catheter inserted into a peripheral vein (n=3)
Cohort 2EXPERIMENTAL2.0 X 10\^14 vg/kg of AVXS-101 delivered one-time through a venous catheter inserted into a peripheral vein (n=12)
Interventions
NameTypeDescription
AVXS-101BIOLOGICALSelf-complementary AAV9 carrying the SMN gene under the control of a hybrid CMV enhancer/chicken-β-actin promoter
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Eligibility Criteria
Age RangeN/A — 6 Months
SexALL
Healthy VolunteersNo
Study Sites1

Inclusion Criteria: * Six or nine months of age and younger (depending on cohort) on day of vector infusion with Type 1 SMA as defined by the following features: * Diagnosis of SMA based on gene mutation analysis with bi-allelic SMN1 mutations (deletion or point mutations) and 2 copies of SMN2. ...

Countries:United States
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