Actively Recruiting

Phase 1
Phase 2
Age: 6Months - 17Years
All Genders
ID05419492

ENDEAVOR: Evaluating ETX101 Gene Therapy Safety and Efficacy in Infants and Children With SCN1A-Positive Dravet Syndrome

Led by Encoded Therapeutics · Updated on 2026-06-03

47

Participants Needed

13

Research Sites

261 weeks

Total Duration

On this page

AI-Summary

What this Trial Is About

Researchers are evaluating ETX101, an investigational gene therapy delivered via AAV9, in infants and children with Dravet syndrome caused by SCN1A gene mutations. This study, called ENDEAVOR, includes participants from 6 months up to less than 18 years old and aims to assess the safety and effects of ETX101 on seizure frequency and developmental outcomes. The trial includes multiple parts with different age groups and study designs to understand its impact comprehensively. The study has three parts: Part 1A is an open-label dose-escalation phase for children aged 6 to less than 36 months, testing up to four dose levels of ETX101. Part 1B is an open-label phase for participants aged 48 months to less than 18 years, evaluating a single dose level. Part 2 is a randomized, double-blind, sham delayed-treatment control study for children aged 6 to less than 48 months, where participants are assigned in a 2:1 ratio to receive ETX101 or a sham procedure with delayed treatment. During participation, children will be monitored for changes in seizure frequency from before dosing to 52 weeks after treatment. Researchers will also assess cognitive and adaptive behavior development using standard scales like Bayley-4 and Vineland-3. Safety and efficacy will be closely observed through clinical evaluations, with follow-up lasting up to a year post-dosing to measure treatment outcomes and monitor health.

CONDITIONS

Brief Title

A Clinical Study to Evaluate the Safety and Efficacy of ETX101 in Infants and Children With SCN1A-Positive Dravet Syndrome

Who Can Participate

Age: 6Months - 17Years
All Genders

Eligibility Criteria

Eligible

You may qualify if you...

  • Participant is between 6 months and less than 36 months old for Part 1A, between 48 months and less than 18 years old for Part 1B, or between 6 months and less than 48 months old for Part 2.
  • Participant has a loss of function pathogenic or likely pathogenic SCN1A gene variant.
  • Participant experienced their first seizure between 3 and 15 months of age.
  • Participant has a clinical diagnosis of Dravet syndrome or a treating clinician suspects Dravet syndrome.
  • Participant is receiving at least one preventive antiseizure medication.
Not Eligible

You will not qualify if you...

  • Participant has another genetic mutation or clinical condition that could affect typical Dravet syndrome symptoms.
  • Participant has a known brain structural or vascular abnormality as shown by MRI or CT scan.
  • Participant has any abnormality interfering with cerebrospinal fluid distribution or has a ventriculoperitoneal shunt.
  • Participant has used sodium channel blockers during the pre-dosing seizure period.
  • Participant has been seizure-free for 4 consecutive weeks within 90 days before consent.
  • Participant has previously received gene or cell therapy.
  • Participant is currently enrolled in another clinical trial or receiving an investigational therapy.
  • Participant has significant liver disease.

AI-Screening

AI-Powered Screening

Complete this quick 3-step screening to check your eligibility

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Your Study Journey

Screening

Duration - 2 to 4 weeks

Participants are screened for eligibility to participate in the trial.

1 visit (in-person)

Treatment

Duration - Week 0 (day of administration)

Participants receive a one-time administration of the ETX101 gene therapy or a sham procedure, depending on randomization.

1 treatment visit (in-person)

Post-treatment Assessment Period

Duration - Week 5 to Week 52

Participants are monitored for safety and efficacy outcomes, including seizure frequency and developmental assessments.

Regular visits through Week 52

Trial Site Locations

Total: 13 locations

1

UCSF Benioff Children's Hospitals

San Francisco, California, United States, 94158

Actively Recruiting

2

Colorado Children's Hospital

Aurora, Colorado, United States, 80045

Not Yet Recruiting

3

Nicklaus Children's Hospital

Miami, Florida, United States, 33155

Actively Recruiting

4

Ann & Robert H. Lurie Children's Hospital of Chicago

Chicago, Illinois, United States, 60611

Actively Recruiting

5

Boston Children's Hospital

Boston, Massachusetts, United States, 02115

Not Yet Recruiting

6

Mott Children's Hospital

Ann Arbor, Michigan, United States, 48109

Not Yet Recruiting

7

Mayo Clinic

Rochester, Minnesota, United States, 55905

Not Yet Recruiting

8

Nationwide Children's Hospital

Columbus, Ohio, United States, 43205

Not Yet Recruiting

9

Oregon Health and Science University (OSHU)

Portland, Oregon, United States, 97239

Actively Recruiting

10

Cook Children's Medical Center

Fort Worth, Texas, United States, 76104

Actively Recruiting

11

The Royal Children's Hospital

Melbourne, Australia

Actively Recruiting

12

Queen Elizabeth Hospital

Glasgow, United Kingdom, G51 4TF

Actively Recruiting

13

Great Ormond Street Hospital

London, United Kingdom, WC1N3JH

Not Yet Recruiting

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Research Team

E

Encoded Patient Advocacy

How is the study designed?

Study Type

INTERVENTIONAL

Masking

QUADRUPLE

Allocation

RANDOMIZED

Model

PARALLEL

Primary Purpose

TREATMENT

Number of Arms

2

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