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rAAVrh74.MHCK7.DYSF.DV

Phase 1

Dysferlinopathy | Small molecule | Other |Sarepta Therapeutics, Inc.|Last Updated: May 13, 2021

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
Double-BlindCONTROLLEDDMCBiomarker
Total Trials1
Total Enrollment2
FDA Designations
No designations recorded
Clinical Trials (1)
NCT IDTitlePhaseStatusEnrollmentVelocityDesignStartCompletionLast UpdatedSitesCountries
NCT02710500rAAVrh74.MHCK7.DYSF.DV for Treatment of DysferlinopathiesPHASE1 COMPLETED 2Mar 1, 2016Jul 1, 2019May 13, 20211 United States
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Study Endpoints
Primary Endpoints
Determination of safety based on the development of unacceptable toxicity
2 Years

Defined as the occurrence of any one Grade III or higher, unanticipated, treatment-related toxicity

Secondary Endpoints
Number of participants showing dysferlin protein expression in muscle tissue
2 Years
Leukocyte marker counts including CD45, CD3, CD4, CD8, and MAC 387.
2 Years
Binding antibodies counts and ELISpot counts to both rAAVrh74 capsid and dysferlin protein.
2 Years
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Study Design & Arms
AllocationNON_RANDOMIZED
MaskingQUADRUPLE
ModelSINGLE_GROUP
PurposeTREATMENT
Treatment Arms
ArmTypeDescription
Cohort 1 (Low Dose)EXPERIMENTALThree (n=3) dysferlin deficiency subjects will receive bilateral injections with one extensor digitorum brevis muscle (EDB) receiving the rAAVrh74.MHCK7.DYSF.DV and the other side receiving saline alone.Subjects will receive a total dose of 2 x 10\^12 in one muscle. Intervention Drug: rAAVrh.MHCK7.DYSF.DV
Cohort 2 (High Dose)EXPERIMENTALThree (n=3) dysferlin deficiency subjects will receive bilateral injections with one extensor digitorum brevis muscle (EDB) receiving the rAAVrh74.MHCK7.DYSF.DV and the other side receiving saline alone.Subjects will receive a total dose of 6 x 10\^12 vg in one muscle. Intervention Drug: rAAVrh74.MHCK7.DYSF.DV
Interventions
NameTypeDescription
rAAVrh74.MHCK7.DYSF.DVDRUGBiological/Vaccine: rAAVrh74.MHCK7.DYSF.DV Recombinant adeno-associated virus carrying a dysferlin transgene under control of a muscle specific MHCK7 promoter.
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Eligibility Criteria
Age Range18 Years — N/A
SexALL
Healthy VolunteersNo
Study Sites1

Inclusion Criteria: * Must be Non-ambulant (cannot walk 10 meters in ≤ 30 sec) and age 18 years or older * Established mutations of the dysferlin gene on both alleles * Impaired muscle function but with sufficient muscle preservation to ensure muscle transfection based on magnetic resonance image o...

Countries:United States
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