RecruitingPhase 2NCT05996003

NS-089/NCNP-02-201 in Boys With Duchenne Muscular Dystrophy (DMD)

A Phase 2 Study to Assess the Efficacy, Safety, Tolerability, and Pharmacokinetics of NS-089/NCNP-02 in Boys With Duchenne Muscular Dystrophy (DMD)


Sponsor

NS Pharma, Inc.

Enrollment

20 participants

Start Date

Feb 22, 2024

Study Type

INTERVENTIONAL

Conditions

Summary

This is a Phase 2, open-label, multi-center, 2-part study of NS-089/NCNP-02 administered by weekly IV infusion to ambulant boys aged ≥4 to \<15 years with DMD due to mutations amenable to exon 44 skipping. Participants will receive a selected dose of NS-089/NCNP-02 administered once weekly. The study consists of 2 parts: Part 1 and Part 2. Six participants (Cohort 1) will participate in both Part 1 and Part 2, and 14 participants (Cohort 2) will be added for Part 2.


Eligibility

Sex: MALEMin Age: 4 YearsMax Age: 14 Years

Inclusion Criteria5

  • Male ≥ 4 years and \<15 years of age
  • Confirmed DMD mutation(s) in the dystrophin gene that is amenable to skipping of exon 44 to restore the dystrophin mRNA reading frame
  • Able to walk independently without assistive devices
  • Ability to complete the TTSTAND without assistance in \<20 seconds
  • Stable dose of glucocorticoid for at least 3 months and the dose is expected to remain on a stable dose for the duration of the study.

Exclusion Criteria7

  • Has a body weight of \<20 kg at the time of informed consent (applies to participants screening for Part 1 only)
  • Evidence of symptomatic cardiomyopathy
  • Current or previous treatment with anabolic steroids (e.g., oxandrolone) or products containing resveratrol or adenosine triphosphate within 3 months prior to first dose of study drug
  • Current or previous treatment with any other investigational drug within 3 months prior to the first dose of study drug or within 5 times the half-life of a medication, whichever is longer
  • Surgery within the 3 months prior to the first dose of study drug or planned during the study duration
  • Previously treated in an interventional study of NS-089/NCNP-02
  • Having received exon skipping oligonucleotide within 1 year prior to the first dose of IP

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Interventions

DRUGNS-089/NCNP-02

Cohort 1: Part 1 Dose Level 1-3: a 4-week Treatment Phase at each treatment dose level Part 2 Single Dose Level: a 24-week Treatment Phase at the MTD of Part 1 Cohort 2: Part 2 Single Dose Level: a 24-week Treatment Phase at the MTD of Part 1


Locations(25)

Children's Hospital Colorado

Aurora, Colorado, United States

Rare Disease Research, LLC - FL

Kissimmee, Florida, United States

Rare Disease Research

Atlanta, Georgia, United States

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

Chicago, Illinois, United States

University of Kansas Medical Center (KUMC)

Kansas City, Kansas, United States

Cincinnati Children's Hospital Medical Center

Cincinnati, Ohio, United States

The Children's Hospital of Philadelphia (CHOP)

Philadelphia, Pennsylvania, United States

University of Pittsburgh School of Medicine

Pittsburgh, Pennsylvania, United States

UT Southwestern/Children's Health

Dallas, Texas, United States

Virginia Commonwealth University Health System

Richmond, Virginia, United States

Perth Children's Hospital

Nedlands, Western Australia, Australia

Alberta Children's Hospital

Calgary, Alberta, Canada

British Columbia Children's Hospital

Vancouver, British Columbia, Canada

London Health Sciences Centre

London, Ontario, Canada

Fukui Prefectural Hospital

Fukui-shi, Fukui, Japan

National Hospital Organization Nagara Medical Center

Nagara, Gifu-shi, Gifu, Japan

NHO Osaka Toneyama Medical Center

Toyonaka, Osaka, Japan

Shiga General Hospital

Moriyama-shi, Shiga, Japan

National Center of Neurology and Psychiatry

Kodaira, Tokyo, Japan

Starship Children's Hospital

Auckland, New Zealand

Pusan National University Yangsan Hospital

Yangsan, Gyeongsangnam, South Korea

Seoul National University Hospital

Seoul, South Korea

Ankara Bilkent City Hospital

Ankara, Turkey (Türkiye)

Yeditepe University Kosuyolu Hospital

Istanbul, Turkey (Türkiye)

S.B.U. Dr. Behcet uz Pediatric Diseases and Surgery Training and Research Hospital

Izmir, Turkey (Türkiye)

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