First in Human (FIH) Study of ALN-SOD in Adult Participants With Amyotrophic Lateral Sclerosis Associated With Mutation in the SOD1 Gene (SOD1-ALS)

NCT ID: NCT06351592

Last Updated: 2025-09-17

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

RECRUITING

Clinical Phase

PHASE1

Total Enrollment

42 participants

Study Classification

INTERVENTIONAL

Study Start Date

2024-08-28

Study Completion Date

2029-04-26

Brief Summary

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This study is researching an experimental drug called ALN-SOD (called "study drug"). This study is focused on people with amyotrophic lateral sclerosis (ALS) caused by a change in a gene called the superoxide dismutase-1 (SOD1) gene. This type of ALS is known as "SOD1-ALS". This is the first time that ALN-SOD will be given to people. The aim of the study is to see how safe and tolerable the study drug is.

The study is looking at several other research questions, including:

* The effect the study drug has on specific biomarkers, which are substances in the blood or in the fluid that surrounds the brain and spinal cord, known as cerebrospinal fluid (CSF)
* How much study drug is in the blood and in the CSF, at different times
* Whether the body makes antibodies against the study drug (which could make the drug less effective or could lead to side effects)
* What effects the study drug has on ALS symptoms

Detailed Description

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Conditions

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Amyotrophic Lateral Sclerosis (ALS) Mutation in the Superoxide Dismutase-1 (SOD1) Gene

Study Design

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Allocation Method

RANDOMIZED

Intervention Model

SEQUENTIAL

Primary Study Purpose

TREATMENT

Blinding Strategy

QUADRUPLE

Participants Caregivers Investigators Outcome Assessors
No masking during Open-Label Treatment Period after 4-week Double-Blind Treatment Period. This part of the study consists of a crossover design.

Study Groups

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Cohort 1 - Low Dose

Placebo during double-blind treatment period

Group Type EXPERIMENTAL

ALN-SOD

Intervention Type DRUG

Administered by intrathecal (IT) injection

Diluent

Intervention Type OTHER

Administered by IT injection

Placebo (PB)

Intervention Type DRUG

Administered by IT injection

Cohort 2 - Mid Dose

Placebo during double-blind treatment period

Group Type EXPERIMENTAL

ALN-SOD

Intervention Type DRUG

Administered by intrathecal (IT) injection

Diluent

Intervention Type OTHER

Administered by IT injection

Placebo (PB)

Intervention Type DRUG

Administered by IT injection

Cohort 3 - High Dose

Placebo during double-blind treatment period

Group Type EXPERIMENTAL

ALN-SOD

Intervention Type DRUG

Administered by intrathecal (IT) injection

Diluent

Intervention Type OTHER

Administered by IT injection

Placebo (PB)

Intervention Type DRUG

Administered by IT injection

Cohort 4 (Optional) ≤ High Dose

Placebo during double-blind treatment period

Group Type EXPERIMENTAL

ALN-SOD

Intervention Type DRUG

Administered by intrathecal (IT) injection

Diluent

Intervention Type OTHER

Administered by IT injection

Placebo (PB)

Intervention Type DRUG

Administered by IT injection

Interventions

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ALN-SOD

Administered by intrathecal (IT) injection

Intervention Type DRUG

Diluent

Administered by IT injection

Intervention Type OTHER

Placebo (PB)

Administered by IT injection

Intervention Type DRUG

Eligibility Criteria

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Inclusion Criteria

1. Weakness attributable to ALS and a SOD1 mutation that has been previously described as associated with ALS or is considered likely to cause ALS, as defined in the protocol
2. Slow vital capacity (SVC) ≥50% predicted value based on age, gender and height, measured in upright position
3. Body Mass Index (BMI) ≤35 kg/m2 at time of screening
4. If participants are taking riluzole or edaravone, they must be on a stable dose for at least 4 weeks prior to initial dosing visit and are expected to remain at that dose until the end of the study
5. Platelet count \>50,000/microliter
6. Has normal blood pressure readings, as defined in the protocol

Exclusion Criteria

1. Concurrent participation in another interventional clinical trial
2. Has had a tracheostomy
3. Has dementia, as assessed by the investigator
4. Has uncontrolled psychiatric disease, including psychosis, active or recent suicidal ideation, untreated major depression, in the past 30 days
5. Has a medical history of brain or spinal disease/injury that would interfere with the lumbar puncture (LP) process, CSF circulation or safety assessment, as defined in the protocol
6. Presence of an implanted shunt for the drainage of CSF or an implanted central nervous system (CNS) catheter
7. Presents any concern to the study investigator that might confound the results of the study or poses an additional risk to the participant by their participation in the study
8. Was hospitalized (ie, \>24 hours) for any reason other than ALS within 30 days of screening
9. Has received treatment with tofersen within 6 months prior to screening
Minimum Eligible Age

18 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Regeneron Pharmaceuticals

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Clinical Trial Management

Role: STUDY_DIRECTOR

Regeneron Pharmaceuticals

Locations

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Concord Repatriation General Hospital

Concord, New South Wales, Australia

Site Status RECRUITING

Macquarie University

Sydney, New South Wales, Australia

Site Status RECRUITING

Sunshine Coast University Hospital

Birtinya, Queensland, Australia

Site Status RECRUITING

University of Alberta Hospital, Edmonton, Division of Neurology

Edmonton, Alberta, Canada

Site Status RECRUITING

University Hospital - London Health Sciences Centre

London, Ontario, Canada

Site Status RECRUITING

Sunnybrook Research Institute

Toronto, Ontario, Canada

Site Status RECRUITING

Montreal Neurological Institute and Hospital

Montreal, Quebec, Canada

Site Status RECRUITING

Hokkaido University Hospital

Sapporo, Hokkaido, Japan

Site Status RECRUITING

Tokushima University Hospital

Tokushima, Tokushima, Japan

Site Status RECRUITING

Toho University Omori Medical Center

Ōta-ku, Tokyo, Japan

Site Status RECRUITING

Kyoto University Hospital

Kyoto, , Japan

Site Status RECRUITING

Hanyang University Seoul Hospital

Seoul, , South Korea

Site Status RECRUITING

Seoul National University Hospital

Seoul, , South Korea

Site Status RECRUITING

Taipei Veterans General Hospital

Taipei, , Taiwan

Site Status RECRUITING

Countries

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Australia Canada Japan South Korea Taiwan

Central Contacts

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Clinical Trials Administrator

Role: CONTACT

844-734-6643

Other Identifiers

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2023-510344-20-00

Identifier Type: REGISTRY

Identifier Source: secondary_id

ALN-SOD-ALS-2351

Identifier Type: -

Identifier Source: org_study_id

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