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Enrolling by invitationNCT06138743Updated Oct 5, 2026

Study of SRP-1003 in Participants With Type 1 Myotonic Dystrophy

A Phase 1/2 interventional study of SRP-1003 IV Infusion and Placebo IV Infusion in Myotonic Dystrophy 1, sponsored by Sarepta Therapeutics, Inc.. Enrolling by invitation at 35 sites in 11 countries. Open to participants aged 18 Years to 65 Years. Per ClinicalTrials.gov, last updated 2026-10-05.

Sponsored by Sarepta Therapeutics, Inc. · Phase 1/2, Interventional, and Treatment

Updated Oct 5, 2026Now Enrolling by invitationGo to Updates ↓
Phase
Phase 1/2
Study type
Interventional
Enrollment
78
Allocation
Randomized
Ages
18 Years to 65 Years
Sex
All
01

Study summary

This is a phase 1/2a double-blinded, placebo-controlled, dose-escalating study to evaluate the safety, tolerability, pharmacokinetics (PK) and pharmacodynamics of single and multiple ascending doses of SRP-1003 compared to placebo in male and female participants with type 1 myotonic dystrophy (DM1). Participants who have provided written informed consent and met all protocol eligibility requirements will be randomized to receive single (Part 1) or multiple (Part 2) doses of SRP-1003 or placebo.

02

Conditions studied

  • Myotonic Dystrophy 1

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Keywords

  • Myotonic Dystrophy 1
  • SRP-1003
  • ARO-DM1
03

In context

Myotonic Dystrophy

126 studies on the registry are indexed under Myotonic Dystrophy; 50 are open to participants now.

This study's planned enrollment of 78 is above the median of 40 across 67 interventional studies indexed under Myotonic Dystrophy.

Browse Myotonic Dystrophy studies →

Lead sponsor

Sarepta Therapeutics, Inc. is the lead sponsor of 52 studies on the registry; 8 are open to participants now.

Of its 21 completed or terminated interventional studies of FDA-regulated products, 12 (57%) have results posted.

Counted across the registry records on this site, refreshed daily.

04

Who can participate

Ages eligible
18 Years to 65 Years
Sexes eligible
All
Accepts healthy volunteers
No

Eligibility criteria

Key Inclusion Criteria:

  • Genetically confirmed diagnosis of DM1
  • Clinician-assessed signs of DM1 including clinically apparent myotonia
  • Onset of DM1 symptoms occurred after the age of 12 years
  • Walk for at least 10 meters independently at screening
  • Participants of must agree to use highly effective in addition to a condom during the study and for at least 90 days following the end of study or last dose of study drug, whichever is later. Participants must not donate sperm or eggs during the study and for at least 90 days following the end of study or last dose of study drug whichever is later.

Key Exclusion Criteria:

  • Inadequately controlled diabetes
  • Confirmed diagnosis of congenital DM1
  • Uncontrolled hypertension
  • History of tibialis anterior (TA) within 3 months of Day 1 or planning to undergo TA biopsies during the study period
  • cardiac, liver or renal disease
  • infection (seropositive) at screening
  • Seropositive for or at screening
  • Untreated or poorly controlled epilepsy
  • Treatment with anti-myotonia medication within a period of 5 half-lives of the medication prior to screening.
  • Abnormal coagulation parameters at screening including count, , prothrombin time, and activated partial thromboplastin time

Note: Additional inclusion/exclusion criteria may apply per protocol

Terms in these criteria, in plain language(9)

Underlined terms in the criteria open these too. Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.

Childbearing potential

Able to become pregnant: from the first period until menopause, unless permanently unable to through surgery (removal of the womb, or of both ovaries or both fallopian tubes).

Criteria for people of childbearing potential usually ask for a negative pregnancy test and for contraception during the study and for a time after it.

Contraception

Ways of preventing pregnancy. “Highly effective” methods fail less than 1% of the time a year when used correctly — for example an IUD or an implant.

Criteria usually say which methods count and how long to keep using them after the last dose, sometimes for male participants too. Some studies accept a partner’s vasectomy if it was confirmed successful and that person is the participant’s only sexual partner.

Biopsy and archival tissue

A biopsy takes a small piece of tissue to examine. “Archival tissue” is a sample already taken, for example at diagnosis, and kept by the hospital.

Whether a stored sample will do depends on when it was taken, how much there is and what the study needs. Ask whether the study accepts your stored sample or needs a new biopsy.

Clinically significant

Judged by the study doctor to matter for your health or for the study. It is a doctor’s decision, so ask the study team if you are unsure whether something counts.

HIV

The human immunodeficiency virus, which weakens the immune system.

Many studies now accept people whose HIV is controlled on treatment, judged by the viral load and the CD4 count. Check the limits this study sets.

Hepatitis B tests

Hepatitis B is a virus that infects the liver. These are separate tests: HBsAg positive usually means a current infection (a recent vaccination can briefly cause it too); total anti-HBc (HBcAb) positive means an infection now or in the past; HBV DNA measures how much virus is in the blood.

Some studies accept a past infection if HBV DNA is undetectable, sometimes with antiviral medicine during the study. Check what this study allows.

Hepatitis C tests

Hepatitis C is a virus that infects the liver. An HCV antibody test can stay positive after an infection has cleared (and is occasionally positive without one); an HCV RNA test shows whether the virus is there now.

After treatment, hepatitis C counts as cured when HCV RNA is still undetectable 12 weeks or more after the treatment ended. Studies often accept people cured this way.

Platelets

Blood cells that help blood clot. Too few raises the risk of bleeding and bruising. A usual adult range is about 150–400 × 10⁹/L (150,000–400,000 per µL); each lab has its own reference range.

INR, PT and aPTT (clotting tests)

Blood tests of how long blood takes to clot. The INR is the prothrombin time (PT) put on a common scale; about 1 is usual for someone not taking a blood thinner.

Criteria often make an exception for people on a blood thinner such as warfarin, whose INR is raised on purpose.

05

Study design

Phase
Phase 1 / Phase 2
Primary purpose
Treatment
Allocation
Randomized
Intervention model
Parallel assignment
Masking
Quadruple (Participant, Care provider, Investigator, Outcomes assessor)
Enrollment
78 participants (estimated)

Study arms

  • Experimental
    SRP-1003 IV Infusion

    Single or multiple doses of SRP-1003 by intravenous (IV) infusion

    Drug: SRP-1003 IV Infusion

  • Placebo comparator
    Placebo by IV Infusion

    Single or multiple doses of placebo by IV infusion

    Drug: Placebo IV Infusion

  • Experimental
    SRP-1003 SC Injection

    Single or multiple doses of SRP-1003 by subcutaneous (SC) injection

    Drug: SRP-1003 SC Injection

  • Placebo comparator
    Placebo by SC Injection

    Single or multiple doses of placebo by SC injection

    Drug: Placebo SC Injection

Interventions

  • DrugSRP-1003 IV Infusion

    SRP-1003 by IV infusion

    Also known as: ARO-DM1 IV Infusion

  • DrugPlacebo IV Infusion

    0.9% sodium chloride (NaCl) calculated volume to match active treatment by IV infusion

  • DrugSRP-1003 SC Injection

    SRP-1003 by SC injection(s)

    Also known as: ARO-DM1 SC Injection

  • DrugPlacebo SC Injection

    0.9% NaCl calculated volume to match active treatment by SC injection(s)

06

What researchers measure

Primary outcomes

  1. Number of Participants with Treatment-emergent Adverse Events Over Time Through End of Study (EOS)

    Time frame: Single-dose phase (Part 1): Up to Day 90 (EOS); multiple-dose phase (Part 2): Up to Day 180 (EOS)

Secondary outcomes

  1. PK of SRP-1003: Maximum Observed Plasma Concentration (Cmax)

    Time frame: Single-dose phase (Part 1): Up 24 hours post-dose; multiple-dose phase (Part 2): Through 24 hours post first and second dose

  2. PK of SRP-1003: Area Under the Plasma Concentration Versus Time Curve from Zero to 24 Hours (AUC0-24)

    Time frame: Single-dose phase (Part 1): Up 24 hours post-dose; multiple-dose phase (Part 2): Through 24 hours post first and second dose

  3. PK of SRP-1003: Area Under the Plasma Concentration Versus Time Curve from Zero to the Last Quantifiable Plasma Concentration (AUClast)

    Time frame: Single-dose phase (Part 1): Up 24 hours post-dose; multiple-dose phase (Part 2): Through 24 hours post first and second dose

  4. PK of SRP-1003: Area Under the Plasma Concentration Versus Time Curve from Zero to Infinity (AUCinf)

    Time frame: Single-dose phase (Part 1): Up 24 hours post-dose; multiple-dose phase (Part 2): Through 24 hours post first and second dose

  5. Change from Baseline at Day 120 for Video Hand Opening Time (vHOT)

    Time frame: (Part 2): Baseline, Day 120

  6. Change from Baseline Over Time for the Timed Up and Go Test (TUG) Assessment

    Time frame: (Part 2): Baseline through EOS (up to 180 days)

  7. Change from Baseline Over Time for the 10-Meter Walk/Run Test (10MWT) Assessment

    Time frame: (Part 2): Baseline through EOS (up to 180 days)

  8. Change from Baseline Over Time for the Hand-held Quantitative Dynamometry Assessment

    Time frame: (Part 2): Baseline through EOS (up to 180 days)

  9. Change from Baseline Over Time for the Video Hand Opening Time (vHOT) Assessment

    Time frame: (Part 2): Baseline through EOS (up to 180 days)

  10. Change from Baseline Over Time for the Myotonic Dystrophy Type 1 Activity and Participation Scale (DM1-Activ-C) Assessment

    Time frame: (Part 2): Baseline through EOS (up to 180 days)

  11. Change from Baseline Over Time for the Myotonic Dystrophy Health Index (MDHI) Assessment

    Time frame: (Part 2): Baseline through EOS (up to 180 days)

07

Study locations

35 sites
08

References and documents

Individual participant data

Plan to share: No

No publications or documents are linked to this record.

09

Timeline

1 registry update since Sep 25, 2026
ElapsedEstimatedEstimated, not yet reached

Started Mar 2024 (actual). Primary completion is estimated for Dec 2026, 33 months after the start.

How these figures are worked out, and which studies are left out of them.

Registered
Nov 14, 2023
First posted
Nov 18, 2023
StartActual
Mar 4, 2024
Primary completionEst.
Dec 31, 2026
CompletionEst.
Dec 31, 2026
Last update
Oct 5, 2026
What these dates mean
Start.
When the first participant enrolled; while estimated, when the study expects to open to recruitment.
Primary completion.
When the last participant was examined or treated to collect data for the study's main question, its primary outcome. A study that stopped early gives the date its data collection stopped.
Completion.
When the last participant's last visit took place, for every outcome, secondary ones and side effects included: the end of the study. Often later than primary completion, sometimes the same day.
Actual and Est.
Actual means the registry says the date has happened. Est. (estimated) is a date the sponsor plans or expects. It can still move, and the sponsor is expected to change it to actual once it has passed.
Status
Recruiting→Enrolling by invitation
changed Oct 5, 2026
Contact
No study contact listed any more: removed S•••@sarepta.com · •••3782
Oct 5, 2026
Show all 1 update
  1. Oct 5, 2026
    Recruiting→Enrolling by invitation
    Contact No study contact listed any more: removed S•••@sarepta.com · •••3782
    + 3 other changes: identifiers, verification date and site details

From the registry record's own update history. This site started tracking changes on Sep 25, 2026; for anything earlier, see the record history on ClinicalTrials.gov ↗

10

Registry details

Key details

Study ID
NCT06138743
Responsible party
Sponsor
First posted
Nov 18, 2023
Start date
Mar 4, 2024
Primary completion
Dec 31, 2026 (estimated)
Completion
Dec 31, 2026 (estimated)
Last update
Oct 5, 2026

Study contacts

Medical Director
study director · Sarepta Therapeutics, Inc.

Oversight

Data monitoring committee
No
FDA-regulated drug
Yes
FDA-regulated device
No
View the source record on ClinicalTrials.gov ↗

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