Gene therapy aims to tame rare metabolic disorder

NCT ID NCT05345171

First seen Jun 27, 2026 · Last updated Jun 27, 2026

Summary

This Phase 3 trial tests a gene therapy called DTX301 for people with late-onset OTC deficiency, a rare genetic disorder that causes dangerous ammonia buildup. The study involves 37 participants and compares the gene therapy to a placebo. The goal is to see if the treatment can help control ammonia levels and reduce the number of metabolic crises.

What this could mean

Our plain-language read of the trial. This is informational only — not medical advice or a prediction.

Active substance

DTX301 (avalotcagene ontaparvovec) gene therapy

What this could lead to

If successful, this gene therapy could help people with OTC deficiency maintain safe ammonia levels and reduce the risk of dangerous metabolic crises.

What could go wrong

This is an early-stage Phase 3 trial with only 37 participants. The therapy may not work for everyone, and side effects from the gene vector or required steroids are possible.

Disclaimer Read more

This is a summary of the original study . Summaries may miss details or leave out important information. Before applying or accepting participation, make sure you have read and understood the full study. Curemydisease.com takes no responsibility whatsoever for anything missed, misunderstood, or acted upon as a result of our summary — we know it does not capture everything.

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Conditions

The condition(s) this trial relates to.

ornithine carbamoyltransferase deficiency

As listed by the trial registrant

The condition terms exactly as the trial's registrant entered them.

Contacts and locations

Locations

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

    Chicago, Illinois, 60611, United States

  • Centro Hospitalar Universitario de Sao Joao

    Porto, 4200-319, Portugal

  • Clinica Universitaria Reina Fabiola

    Córdoba, X5004, Argentina

  • Erasmus Universitair Medisch Centrum Rotterrdam

    Rotterdam, 3015, Netherlands

  • Fujita Health University Hospital

    Toyoake, 470-1192, Japan

  • Fundacio Hospital Universitari Vall D'Hebron-Institute de Recerca

    Barcelona, 08035, Spain

  • Hopital Femme Mere Enfant

    Bron, 69500, France

  • Hospital Italiano de Buenos Aires

    Buenos Aires, C1199, Argentina

  • Hospital de Clinicas de Porto Alegre

    Porto Alegre, 90035-903, Brazil

  • Kumamoto University Hospital

    Kumamoto, 860-8556, Japan

  • Necker-Enfants Maladas Hospital

    Paris, 75015, France

  • The Hospital for Sick Children

    Toronto, Ontario, M5G 1X8, Canada

  • Universitatsklinikum Heidelberg

    Heidelberg, 69120, Germany

  • University Hospitals Cleveland Medical Center

    Cleveland, Ohio, 44106, United States

  • University of California

    Los Angeles, California, 90095, United States

  • University of Colorado

    Aurora, Colorado, 80045, United States