AMX0114 in Adult Participants With Amyotrophic Lateral Sclerosis

NCT ID: NCT06665165

Last Updated: 2025-10-09

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

48 participants

Study Classification

INTERVENTIONAL

Study Start Date

2025-04-07

Study Completion Date

2027-10-31

Brief Summary

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This study is a placebo-controlled Phase I study to evaluate the safety, tolerability, pharmacokinetics, and pharmacodynamics of the antisense oligonucleotide (ASO) AMX0114 in adult participants with amyotrophic lateral sclerosis (ALS).

Detailed Description

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The purpose of this study is to determine how safe and tolerable the investigational drug, AMX0114, is for the treatment of amyotrophic lateral sclerosis (ALS).

AMX0114 is given by intrathecal injection, an injection in the lower back into the spinal canal, also known as lumbar puncture. This clinical trial is designed to test if the treatment is safe and tolerable by monitoring the incidence of adverse events, serious adverse events, dose limiting toxicities (DLTs), and incidence of abnormalities in clinical laboratory assessments, vital signs, physical and neurological examinations, and electrocardiograms (ECGs). This trial will also assess the effects of AMX0114 on biomarkers of ALS, including markers of neuronal death and neuroinflammation.

Conditions

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ALS

Study Design

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Allocation Method

RANDOMIZED

Intervention Model

SEQUENTIAL

Primary Study Purpose

TREATMENT

Blinding Strategy

QUADRUPLE

Participants Caregivers Investigators Outcome Assessors

Study Groups

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Active Treatment: AMX0114

AMX0114 will be administered once every 4 weeks by intrathecal bolus injection for a total of up to 4 doses. Treatment will be administered on Day 1, followed by repeat dosing every 4 weeks at approximately Day 29, Day 57 and Day 85.

Group Type EXPERIMENTAL

AMX0114

Intervention Type DRUG

Antisense oligonucleotides (ASOs) are a type of medicine that treats diseases by intercepting the mRNA messages sent within the cell, resulting in fewer specific proteins being made. AMX0114 is an ASO that targets the mRNA messenger that instructs the body to create a protein called calpain-2. Calpain-2 has been linked to the degeneration and death of neurons in many neurological diseases, including people living with sporadic ALS. AMX0114 is designed to reduce the levels of calpain-2, with the goal of slowing down the process that leads to neuron injury and death.

Placebo

Placebo drug will be administered once every 4 weeks by intrathecal bolus injection for a total of up to 4 doses. Treatment will be administered on Day 1, followed by repeat dosing every 4 weeks at approximately Day 29, Day 57 and Day 85.

Group Type PLACEBO_COMPARATOR

Placebo

Intervention Type OTHER

Placebo

Interventions

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AMX0114

Antisense oligonucleotides (ASOs) are a type of medicine that treats diseases by intercepting the mRNA messages sent within the cell, resulting in fewer specific proteins being made. AMX0114 is an ASO that targets the mRNA messenger that instructs the body to create a protein called calpain-2. Calpain-2 has been linked to the degeneration and death of neurons in many neurological diseases, including people living with sporadic ALS. AMX0114 is designed to reduce the levels of calpain-2, with the goal of slowing down the process that leads to neuron injury and death.

Intervention Type DRUG

Placebo

Placebo

Intervention Type OTHER

Eligibility Criteria

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Inclusion Criteria

1. Ability to understand the purpose and risks of this study, willingness to comply with the study and to provide informed consent in accordance with local laws and regulations.
2. Male or female, at least 18 years of age.
3. Diagnosis of clinically definite or clinically probable ALS, made by a physician who is experienced with management of ALS.
4. Time since onset of first symptom of ALS should be \<24 months prior to beginning the study. Date of ALS symptom onset is defined as the onset of weakness (in the limbs, bulbar region, or trunk).
5. If the participant is to be treated with riluzole and/or edaravone before or during the trial, then treatment must be previously started and maintained at a stable regimen for at least 30 days prior to starting the study and through the end of the study.
6. Women of childbearing potential (e.g., not post-menopausal for at least one year or surgically sterile) must agree to use an acceptable birth control method for the duration of the trial and 60 days after the last dose of Study Drug or be of non-childbearing potential.
7. Female participants or female partners of male participants must not be pregnant or plan to become pregnant for the duration of the trial and for up to 90 days after the last dose of Study Drug.
8. Male participants must agree to abstain from sperm donation for the duration of the trial and practice contraception with a female partner, for at least 90 days after last dose of Study Drug.

Exclusion Criteria

1. Presence of tracheostomy or permanent assisted ventilation.
2. SVC less than 75%.
3. Abnormal liver function defined as aspartate aminotransferase and/or alanine aminotransferase \> 3 times the upper limit of normal (ULN) and/or total bilirubin \> 1.5 times the ULN (obtained within 4 weeks of first dose) except when a result of Gilbert syndrome.
4. Abnormal renal function defined as estimated glomerular filtration rate (eGFR) \< 60 mL/min/1.73m2.
5. Other laboratory abnormalities, including abnormalities in platelet count, international normalized ratio, prothrombin time, and activated partial thromboplastin time.
6. Pregnant women (confirmed by a pregnancy test within 7 days prior to first dose) or women currently breastfeeding.
7. Current or previous clinically significant, unstable medical condition (other than ALS), that in the opinion of the Investigator could affect a participant's safety or ability to comply with the study.
8. Significant abnormalities in physical/neurological examination, vital signs, or electrocardiogram (ECG), which in the opinion of the Investigator could affect the safety of the participant.
9. Presence of unstable psychiatric disease, cognitive impairment, dementia or substance abuse that could affect the participant's ability to provide informed consent or comply with study procedures.
10. Current or previous enrollment in another trial involving use of an investigational therapy, in most cases within 30 days after the last dose of the study drug, prior to starting this study.
11. Current or previous treatment with small interfering ribonucleic acid, stem cell therapy, any ASO or gene therapy.
12. Any contraindications for lumbar puncture or repeated intrathecal injection and/or underlying disorders that could be affected by intrathecal injections.
13. Prior severe reaction or known hypersensitivity to any part of the Study Drug.
Minimum Eligible Age

18 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Amylyx Pharmaceuticals Inc.

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Medical Director, Amylyx

Role: STUDY_DIRECTOR

Medical Monitor

Locations

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University of California, San Diego

La Jolla, California, United States

Site Status RECRUITING

Georgetown University Hospital Pasquerilla Healthcare Center

Washington D.C., District of Columbia, United States

Site Status RECRUITING

University of Florida

Gainesville, Florida, United States

Site Status RECRUITING

Mayo Clinic in Florida

Jacksonville, Florida, United States

Site Status RECRUITING

Orlando Regional Medical Center, Orlando Health Neuroscience Institute

Orlando, Florida, United States

Site Status RECRUITING

Massachusetts General Hospital, Healey & AMG Center for ALS

Boston, Massachusetts, United States

Site Status RECRUITING

Mayo Clinic in Rochester

Rochester, Minnesota, United States

Site Status RECRUITING

Temple University of the Commonwealth System of Higher Education

Philadelphia, Pennsylvania, United States

Site Status RECRUITING

Alliance for Multispecialty Research, LLC

Knoxville, Tennessee, United States

Site Status RECRUITING

Houston Methodist Neurological Institute

Houston, Texas, United States

Site Status RECRUITING

University of Calgary

Calgary, Alberta, Canada

Site Status RECRUITING

McMaster University

Hamilton, Ontario, Canada

Site Status RECRUITING

London Health Sciences Centre

London, Ontario, Canada

Site Status RECRUITING

McGill University Health Centre - Centre for Innovative Medicine

Montreal, Quebec, Canada

Site Status RECRUITING

Countries

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United States Canada

Central Contacts

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Medical Director, Amylyx

Role: CONTACT

857-320-6200

Facility Contacts

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Rose Previte

Role: primary

617-697-4856

Cate Ledoux

Role: primary

240-681-9556

Jennifer Steshyn

Role: primary

518-262-5226

Jeffery Gainer

Role: primary

904-244-4693

Charlene Carlo

Role: primary

3218411324

Mia Fleischer

Role: primary

617-724-5659

Vanessa Travero

Role: backup

617-724-2069

Brenda Nelson

Role: primary

507-293-9237

Kathleen Hatala, 215-707-4171

Role: primary

Christine Barr

Role: backup

215-707-5440

Patricia Pryor

Role: primary

865-305-9100

Felicia Hiatt

Role: backup

Jennifer Garrett

Role: primary

634-238-4516

Janet Petrillo Ballantyne

Role: primary

403-210-7006

Daniela Trapsa

Role: primary

905-521-2100 ext. 76368

Denise Hulley

Role: primary

5196858500 ext. 34345

Maria Gobbo

Role: primary

Other Identifiers

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A114-001

Identifier Type: -

Identifier Source: org_study_id

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