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EPI-743

Phase 2

Leigh Syndrome | Small molecule | Other |PTC Therapeutics, Inc.|Last Updated: Oct 23, 2024

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
RandomizedDouble-BlindPLACEBO_CONTROLLEDDMCBiomarker
Total Trials2
Total Enrollment65
FDA Designations
No designations recorded
Clinical Trials (2)
NCT IDTitlePhaseStatusEnrollmentVelocityDesignStartCompletionLast UpdatedSitesCountries
NCT02352896Long-Term Safety and Efficacy Evaluation of EPI-743 in Children With Leigh SyndromePHASE2 COMPLETED 30Jan 31, 2014Oct 30, 2023Oct 23, 20244 United States
NCT01721733Safety and Efficacy Study of EPI-743 in Children With Leigh SyndromePHASE2 COMPLETED 35Oct 31, 2012May 31, 2015Aug 31, 20204 United States
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Study Endpoints
Primary Endpoints
Disease Severity as Measured by Newcastle Pediatric Mitochondrial Disease Scale (NPMDS) Sections 1-3 Score
Baseline up to Month 36

NPMDS is a validated scale to assess the mitochondrial disease progression.

Number of Participants With Dose-Limiting Serious Adverse Events (SAEs)
Baseline up to Month 36
Newcastle Pediatric Mitochondrial Disease Scale (NPMDS) Sections 1-3
6 months

Change from baseline to six months will be compared between subjects in active treatment group and placebo group

Secondary Endpoints
Bayley Scales of Infant Development-III Score (Participants Age 0-3)
Baseline up to Month 24
Wechsler Scale of Intelligence and Movement Assessment Battery Score for Children (Participants Age 4-18)
Baseline up to Month 24
Barry-Albright Dystonia Scale Score
Baseline up to Month 24
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Study Design & Arms
AllocationNA
MaskingNONE
ModelSINGLE_GROUP
PurposeTREATMENT
Treatment Arms
ArmTypeDescription
EPI-743EXPERIMENTALParticipants will receive EPI-743 at a dose of 15 milligrams/kilogram (mg/kg) up to a total 200 mg 3 times daily (TID) orally with meal or by using nasogastric (NG) or gastrostomy feeding tubes with liquid food for at least 36 months.
PlaceboPLACEBO_COMPARATOREach patient will receive a volume of placebo based on weight
EPI-743 15 mg/kgACTIVE_COMPARATOREach subjects dose will be based on their weight. 15 mg/kg with a maximum dose of 200 mg per dose, t.i.d., will be administered in this treatment arm.
EPI-743 5 mg/kgACTIVE_COMPARATOREach subjects dose will be based on their weight. 5 mg/kg with a maximum dose of 100 mg per dose, t.i.d., will be administered in this treatment arm.
Interventions
NameTypeDescription
EPI-743DRUGEPI-743 100 milligrams/milliliter (mg/mL) oral solution will be administered per dose and schedule specified in the arm.
PlaceboDRUG -
EPI-743 15 mg/kgDRUG -
EPI-743 5 mg/kgDRUG -
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Eligibility Criteria
Age Range1 Year — 18 Years
SexALL
Healthy VolunteersNo
Study Sites4

Inclusion Criteria: 1. Diagnosis of Leigh syndrome with genetic confirmation 2. Completion of EPI743-12-002 protocol and initiation of treatment within 14 days of Month 12 visit in EPI743-12-002 study 3. Participant or participant's guardian able to consent and comply with protocol requirements 4. ...

Countries:United States
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