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Duchenne Muscular DystrophyPhase 3ESSENCENCT02500381

Study of SRP-4045 (Casimersen) and SRP-4053 (Golodirsen) in Participants With Duchenne Muscular Dystrophy (DMD)

A Phase 3 study testing SRP-4045 and SRP-4053 in people with Duchenne Muscular Dystrophy, run by Sarepta Therapeutics, Inc. at 76 sites in 24 countries.

Completed

Finished. The study has ended and is not enrolling. Results may be posted on the official record.

Official record
Search all trials

Trial summary

The main objective of this study is to evaluate the efficacy of SRP-4045 (casimersen) and SRP-4053 (golodirsen) compared to placebo in participants with DMD with out-of-frame deletion mutations amenable to skipping exon 45 and exon 53, respectively. Read more about this study

Who is it for?
Ages 6 to 13 years, men only
Conditions the study is for
Duchenne Muscular Dystrophy
What is being tested?
SRP-4045 and SRP-4053
Type of treatment
Injection or infusion
Could I receive a placebo?
Yes, some participants may receive a placebo
How many people
228 participants
Where
76 sitesin 24 countries
Study dates
28 Sep 2016to 16 Oct 2025
Phase
Phase 3
Are there results?
None posted in the registry

Key requirements

Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.

  • Ages 6 to 13 years
  • Men only
  • No healthy volunteers
  • Child

Who may be able to join

  • Genotypically confirmed DMD, with genetic deletion amenable to exon 45 or exon 53 skipping
  • Stable dose of oral corticosteroids for at least 24 weeks prior to Week 1, and the dose is expected to remain constant throughout the study (except for modifications to accommodate changes in weight).
  • Intact right and left biceps brachii muscles or 2 alternative upper arm muscle groups
  • Mean 6MWT ≥300 meters and ≤450 meters
  • Stable pulmonary function: forced vital capacity (FVC) ≥50% predicted

Who cannot join

  • Treatment with gene therapy at any time
  • Previous treatment with SMT C1100 within 1 week prior to Week 1 and previous treatment with PRO045 (BMN 045), PRO053 (BMN 053), or PRO051 (BMN 051) within 24 weeks prior to Week 1
  • Current or previous treatment with any other experimental treatment within 12 weeks prior to Week 1
  • Major surgery within 3 months prior to Week 1
  • Presence of other clinically significant illness
  • Other inclusion/exclusion criteria may apply.

Trial locations and contacts

76 sites in 24 countries.

76 sites in 24 countries

United States23 sites
  • Center for Integrative Rare Disease Research (CIRDR)

    Atlanta, Georgia, 30318

  • Boston Children's Hospital

    Boston, Massachusetts, 02115

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

    Chicago, Illinois, 60611

  • Cincinnati Children's Hospital Medical Center (CCHMC)

    Cincinnati, Ohio, 45229

  • Nationwide Children's Hospital

    Columbus, Ohio, 43205

  • Children's Medical Center Dallas

    Dallas, Texas, 75235

  • University of Florida

    Gainesville, Florida, 32610

  • NW Florida Clinical Research Group, LLC

    Gulf Breeze, Florida, 32561

  • University of Iowa Children's Hospital

    Iowa City, Iowa, 52242

  • University of Kansas, Medical Center

    Kansas City, Kansas, 66160

  • Las Vegas Clinic

    Las Vegas, Nevada, 89145

  • Children's Hospital Los Angeles

    Los Angeles, California, 90027

  • David Geffen School of Medicine, UCLA

    Los Angeles, California, 90095

  • Children's Hospital of Wisconsin

    Milwaukee, Wisconsin, 53226

  • Children's Hospital of the King's Daughters

    Norfolk, Virginia, 23507

  • Neuromuscular Research Center

    Phoenix, Arizona, 85028

  • Children's Hospital of Pittsburgh of UPMC

    Pittsburgh, Pennsylvania, 15224

  • Shriners Hospital for Children

    Portland, Oregon, 97239

  • University of Rochester Clinical Research Center

    Rochester, New York, 14642

  • University of Utah

    Salt Lake City, Utah, 84132

  • Rady Children's Hospital San Diego/ UCSD

    San Diego, California, 92123

  • St. Louis Children's Hospital

    St Louis, Missouri, 63110

  • Stanford University School of Medicine/Medical Center

    Stanford, California, 94305

Other trials in United States

United Kingdom6 sites
  • Royal Hospital for Children (Glasgow)

    Glasgow, G51 4TF

  • Leeds Teaching Hospitals NHS Trust

    Leeds, LS1 3EX

  • Alder Hey Childrens Hospital

    Liverpool, L12 2AP

  • Great Ormond Street Hospital (GOSH)

    London, WC1N 1EH

  • Royal Victoria Infirmary

    Newcastle upon Tyne, NE1 4LP

  • John Radcliffe Hospital

    Oxford, OX3 9DU

Other trials in United Kingdom

Italy5 sites
  • Azienda Ospedaliero-Universitaria di Ferrara - Arcispedale Sant' Anna

    Ferrara, 44124

  • Istituto Giannina Gaslini

    Genoa, 16147

  • Az Ospedaliera Universitaria Policlinico G Martino

    Messina, 98125

  • Fondazione IRCCS Istituto Neurologico Carlo Besta

    Milan, 20133

  • Policlinico Universitario A Gemelli

    Rome, 00168

Other trials in Italy

Canada4 sites
  • Alberta Childrens Hospital

    Calgary, Alberta, T3B 6A8

  • London Health Sciences Centre

    London, Ontario, N6A 5W9

  • Children's Hospital of Eastern Ontario

    Ottawa, Ontario, K1H 8L1

  • Children's and Women's Health Centre of British Columbia

    Vancouver, British Columbia, V6H 3V4

Other trials in Canada

Germany4 sites
  • Charité - Universitätsmedizin Berlin

    Berlin, 13353

  • Universitätsklinikum Essen

    Essen, 45122

  • University Hospital Freiburg

    Freiburg im Breisgau, 79106

  • LMU Klinikum der Universität

    München, Bavaria, 80337

Other trials in Germany

Australia3 sites
  • Royal Children's Hospital Melbourne

    Parkville, Victoria, 3052

  • Queensland Children's Hospital

    South Brisbane, 4101

  • Children's Hospital at Westmead

    Westmead, 2145

Other trials in Australia

Belgium3 sites
  • Universitair Ziekenhuis Gent

    Ghent, 9000

  • Universitair Ziekenhuis Leuven

    Leuven, 3000

  • CHR de la Citadelle

    Liège, 4000

Other trials in Belgium

France3 sites
  • Reference Centre for Neuromuscular Diseases

    Nantes, 44093

  • Hôpital Armand Trousseau

    Paris, 75012

  • Hôpital Des Enfants

    Toulouse, Haute-Garonne, 31059

Other trials in France

Spain3 sites
  • Hospital de La Santa Creu i Sant Pau

    Barcelona, 08041

  • Hospital Sant Joan de Deu

    Barcelona, 08950

  • Hospital Universitari i Politecnic La Fe de Valencia

    Valencia

Other trials in Spain

Czechia2 sites
  • University Hospital Brno

    Brno, 61300

  • Fakultni nemocnice v Motole

    Prague, 15008

Other trials in Czechia

Greece2 sites
  • IASO Children's Hospital

    Marousi, 15123

  • Ippokratio General Hospital of Thessaloniki

    Thessaloniki, 54642

Other trials in Greece

India2 sites
  • Royal Instituite of Child Neurosciences

    Ahmedabad, Gujarat, 380054

  • Deenanth Mangeshkar Hospital

    Pune, Maharashtra, 411004

Other trials in India

Mexico2 sites
  • Neurociencias Estudios Clínicos S.C

    Culiacán, 80020

  • Instituto de Investigaciones Clínicas para la Salud A.C

    Durango, 34000

Other trials in Mexico

Poland2 sites
  • Uniwersyteckie Centrum Kliniczne

    Gdansk, 80-952

  • Samodzielny Publiczny Centralny Szpital Kliniczny

    Warsaw, Masovian Voivodeship, 02-097

Other trials in Poland

Russia2 sites
  • Federal state budget educational institution of higher education "Russian national research medical university n.a. N.I. Pirogov" of Ministry of healthcare of Russian Federation

    Moscow, 125412

  • State Autonomous Healthcare Institution of Sverdlovsk Region Children's Clinical Hospital No. 9 City of Ekaterinburg

    Yekaterinburg

Other trials in Russia

South Korea2 sites
  • Seoul National University Hospital

    Seoul, 03080

  • Pusan National University Yangsan Hospital

    Yangsan, 50612

Other trials in South Korea

Argentina1 site
  • DOM Centro de Reumatologia

    Buenos Aires, 1111

Other trials in Argentina

Bulgaria1 site
  • University Multiprofile Hospital for Active Treatment Aleksandrovska EAD

    Sofia, Sofia-Grad, 1431

Other trials in Bulgaria

Denmark1 site
  • Rigshospitalet Copenhagen University Hospital

    København Ø, 2100

Other trials in Denmark

Hungary1 site
  • Semmelweis Egyetem Genomikai Medicina és Ritka Betegsegek Intezete

    Budapest, 1083

Other trials in Hungary

Ireland1 site
  • The Children's University Hospital

    Dublin, D1

Other trials in Ireland

Israel1 site
  • Schneider Children's Medical Center of Israel

    Petah Tikvah, 49102

Other trials in Israel

Serbia1 site
  • Clinic for Neurology and Psychiatry for Children and Youth

    Belgrade, 11000

Other trials in Serbia

Sweden1 site
  • Drottning Silvias Barn Och Ungdomssjukhus

    Gothenburg, SE-41685

Other trials in Sweden

About this study

Official title: A Double-Blind, Placebo-Controlled, Multi-Center Study With an Open-Label Extension to Evaluate the Efficacy and Safety of SRP-4045 and SRP-4053 in Patients With Duchenne Muscular Dystrophy

The main objective of this study is to evaluate the efficacy of SRP-4045 (casimersen) and SRP-4053 (golodirsen) compared to placebo in participants with DMD with out-of-frame deletion mutations amenable to skipping exon 45 and exon 53, respectively.

Read the detailed description

This is a double-blind, placebo-controlled, multi-center study to evaluate the efficacy and safety of SRP-4045 and SRP-4053. Eligible participants with out-of-frame deletion mutations amenable to exon 45 or 53 skipping will be randomized to receive once weekly intravenous (IV) infusions of 30 milligrams/kilograms (mg/kg) SRP-4045 or 30 mg/kg SRP-4053 respectively (combined-active group) or placebo for up to 96 weeks (the placebo-controlled period of the trial). This will be followed by an open-label extension period in which all participants will receive open-label active treatment for 48 weeks (up to Week 144 of study).

The study will enroll approximately 222 participants. Twice as many participants will be randomized to receive active treatment as will receive placebo (2:1 randomization).

Clinical efficacy will be assessed at regularly scheduled study visits, including functional tests, such as the 6-minute walk test (6MWT). All participants will undergo a muscle biopsy at baseline and a second muscle biopsy either at Week 48 or Week 96.

Safety will be assessed through the collection of adverse events (AEs), laboratory tests, electrocardiograms (ECGs), echocardiograms (ECHOs), vital signs, and physical examinations throughout the study.

Blood samples will be taken periodically throughout the study to assess the pharmacokinetics of both drugs.

Being tested
SRP-4045, SRP-4053, Placebo
Keywords
Duchenne muscular dystrophy, Exon Skipping, DMD, Exon 53, Exon 45, Ambulatory, Pediatric, Duchenne
Medical subject headings
Muscular Dystrophy, Duchenne

What participants receive

What participants receive. In a randomised study each person is assigned to one group by chance.

  • SRP-4045Drug

    Also called Casimersen, AMONDYS 45

    SRP-4045 solution for IV infusion

  • SRP-4053Drug

    Also called Golodirsen, VYONDYS 53

    SRP-4053 solution for IV infusion

  • PlaceboDrug

    SRP-4045 or SRP-4053 placebo-matching solution for IV infusion

Study groups in NCT02500381
Study groupRoleWhat they receive
SRP-4045ExperimentalParticipants amenable to exon 45 skipping will receive SRP-4045 IV infusions, weekly, at 30 mg/kg for up to 96 weeks in the double-blinded period. This will be followed by an open-label extension period in which all participants will receive open-label active treatment of SRP-4045 at 30 mg/kg/week IV infusions for 48 weeks (up to Week 144 in the study).
Receives: Drug: SRP-4045
SRP-4053ExperimentalParticipants amenable to exon 53 skipping will receive SRP-4053 IV infusions, weekly, at 30 mg/kg for up to 96 weeks in the double-blinded period. This will be followed by an open-label extension period in which all participants will receive open-label active treatment of SRP-4053 at 30 mg/kg/week IV infusions for 48 weeks (up to Week 144 in the study).
Receives: Drug: SRP-4053
Placebo followed by SRP-4045 or SRP-4053Placebo comparatorParticipants amenable to exon 45 or 53 skipping will receive SRP-4045 or SRP-4053 placebo-matching IV infusions, weekly, at 30 mg/kg for up to 96 weeks in the double-blinded period. This will be followed by an open-label extension period in which all participants will receive open-label active treatment of SRP-4045 or SRP-4053 at 30 mg/kg/week IV infusions for 48 weeks (up to Week 144 in the study).
Receives: Drug: SRP-4045; Drug: SRP-4053; Drug: Placebo

What is being measured

The primary outcome is the main question the study is designed to answer.

  1. Change From Baseline in the 4-Step Ascend Velocity at Week 96

    Measured: Baseline, Week 96

Secondary outcomes (7)
  1. Change from Baseline in the Total Distance Walked During 6MWT at Week 96

    Measured: Baseline, Week 96

  2. Change from Baseline in Rise from Floor Velocity at Week 96

    Measured: Baseline, Week 96

  3. Change From Baseline in the 4-Step Ascend Velocity at Week 144

    Measured: Baseline, Week 144

  4. Change From Baseline in Total Distance Walked During the 10-meter walk/run (10-MWR) Velocity

    Measured: Baseline, Week 96

  5. Change From Baseline in the North Star Ambulatory Assessment (NSAA) Total Score at Week 96

    The NSAA is a clinician administered scale that rates the participant's performance on various functional activities. During this assessment, participants will be asked to perform 17 different functional activities, including a 10 meter walk/run, rising from a sit to standing, standing on 1 leg, climbing a box step, descending a box step, rising from lying to sitting, rising from the floor, lifting the head, standing on heels, and jumping. Participants will be graded as follows: 2 = achieves goal without any assistance; 1 = modified method but achieves goal independent of physical assistance from another person; and 0 = unable to achieve goal independently. NSAA Total Score ranges from 0 to 34, with a score of 34 implying normal function.

    Measured: Baseline, Week 96

  6. Change from Baseline in Dystrophin Protein Levels Determined by Western Blot at Weeks 48 or 96

    Measured: Baseline, Week 48 or Week 96

  7. Change from Baseline in Dystrophin Intensity Levels Determined by Immunohistochemistry (IHC) at Weeks 48 or 96

    Measured: Baseline, Week 48 or Week 96

Timeline

From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.

  1. First posted16 Jul 2015 (estimated)
  2. Study start28 Sep 2016
  3. Primary completion12 Nov 2024
  4. Study completion16 Oct 2025

Resources

More details

For doctors and researchers: how the study is designed, who runs it, and its registry IDs.

Study design
Study type
Interventional
Phase
Phase 3
Primary purpose
Treatment
Allocation
Randomised
Intervention model
Parallel groups
Masking
Quadruple blind
Who is masked
Participant, Care provider, Investigator, Outcomes assessor
Enrolment
228 participants (actual)
IDs, dates and publications
ClinicalTrials.gov ID
NCT02500381
Sponsor's protocol ID
4045-301
Other IDs
2015-002069-52, 2024-514698-23-00, 2015
Acronym
ESSENCE
Status last verified
Nov 2025
First submitted
14 Jul 2015
Last updated
18 Nov 2025
Results posted
No
Data monitoring committee
Yes
FDA-regulated drug
Yes
FDA-regulated device
No
Individual data sharing
Undecided

Publications

  • Vandekerckhove I, Hanssen B, Peeters N, Dewit T, De Beukelaer N, Van den Hauwe M, De Waele L, Van Campenhout A, De Groote F, Desloovere K. Anthropometric-related percentile curves for muscle size and strength of lower limb muscles of typically developing children. J Anat. 2025 Aug;247(2):348-362. doi: 10.1111/joa.14241. Epub 2025 Mar 17.

    PubMed 40098309
  • Wagner KR, Kuntz NL, Koenig E, East L, Upadhyay S, Han B, Shieh PB. Safety, tolerability, and pharmacokinetics of casimersen in patients with Duchenne muscular dystrophy amenable to exon 45 skipping: A randomized, double-blind, placebo-controlled, dose-titration trial. Muscle Nerve. 2021 Sep;64(3):285-292. doi: 10.1002/mus.27347. Epub 2021 Jun 29.

    PubMed 34105177

Questions about this trial

What is this clinical trial studying?

A Phase 3 study testing SRP-4045 and SRP-4053 in people with Duchenne Muscular Dystrophy, run by Sarepta Therapeutics, Inc. at 76 sites in 24 countries.

The full registered description is in the About this study section and the official record.

Who may be eligible to take part?

The sponsor lists 5 inclusion and 6 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.

Is this clinical trial currently recruiting?

The record says: finished, as of the sponsor's last update on 18 Nov 2025. Status can change between updates, so confirm with the study contact before making plans.

Where is the study taking place?

The record lists 76 sites in United States, United Kingdom, Italy, Canada, Germany and 19 more countries. Whether a site is taking part right now should be confirmed with that site. See every site.

What are the possible benefits and risks?

They depend on the study and the treatment, and no study can promise a benefit. Some participants in this study may receive a placebo. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.

What does this phase mean?

Phase 3 studies compare the treatment with the current standard treatment or a placebo in a large group, to confirm how well it works and watch for side effects.

Not every study follows the same path, and some studies (observational studies, device studies) have no phase.

Are results available for this study?

The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.

How can a doctor refer a patient or contact the research team?

The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.

What does the primary outcome measure?

Change From Baseline in the 4-Step Ascend Velocity at Week 96 (time frame: Baseline, Week 96). It is what the study was designed to measure, not a finding.

Where can I read the original study information?

On the ClinicalTrials.gov record for NCT02500381, which also lists 2 linked publications.

Does a listing mean the treatment is approved or proven to work?

No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.

How often is this information updated?

AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 18 Nov 2025.

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