Acoramidis Transthyretin Amyloidosis Prevention Trial in the Young (ACT-EARLY) Study in Asymptomatic Carriers of a Pathogenic TTR Variant

NCT ID: NCT06563895

Last Updated: 2025-12-30

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

PHASE3

Total Enrollment

582 participants

Study Classification

INTERVENTIONAL

Study Start Date

2025-05-12

Study Completion Date

2032-12-31

Brief Summary

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Transthyretin amyloidosis (ATTR) is a disease where the normally occurring transthyretin (TTR) protein falls apart and forms amyloid, a sticky plaque- like substance that accumulates in different organs in the body and can cause damage to the organ. There are two ways that the TTR protein can fall apart. One way occurs as a person ages, where the normal TTR protein can fall apart and form amyloid that may no longer be sufficiently cleared by the body. This type of ATTR is known as wild-type ATTR (ATTRwt). The other way occurs when a person inherits a defective TTR gene that causes the TTR protein to spontaneously fall apart. This form of the disease is known as variant ATTR (ATTRv) and can be detected in adults by a genetic test of their TTR gene before they age.

Amyloid build-up in the heart causes the heart wall to become thick and stiff and can result in heart failure and even death. Accumulation of TTR amyloid in the heart is known as transthyretin amyloid cardiomyopathy or ATTR-CM. Amyloid can also deposit in the nerve tissues leading to nerve problems. Accumulation of TTR in the nerves is known as transthyretin amyloid polyneuropathy or ATTR-PN.

Acoramidis is an experimental drug designed to bind tightly to TTR in the blood and stabilize its structure, so it does not form the harmful amyloid plaques that can cause damage to organs.

This study is intended to determine if treatment with acoramidis in participants with ATTRv who have not yet developed any symptoms of disease can prevent or delay the development of ATTR-CM or ATTR-PN disease. If adults with an inherited defective TTR gene are treated early before any of the symptoms of disease have developed, it may be possible to delay the onset or prevent the disease entirely.

Detailed Description

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The AG10-501 ACT-EARLY study is a randomized, multicenter, double-blind, placebo- controlled study of acoramidis for prevention of ATTR (with specific reference to either its cardiomyopathic or polyneuropathic manifestations). Participants will be stratified at randomization.

The study population will be asymptomatic carriers of a known pathogenic TTR gene variant. A participant must be 18 to 75 inclusive years of age, and the age of the participant must be no more than 10 years younger than the predicted age of disease onset (PADO) based either on family history (pedigree analysis) or, if family history is insufficient, based on a TTR Variant Actuarial table from published literature. For example, if PADO for a given individual is found to be 50 years, the age of the participant must be between 40 and 75 years inclusive.

Conditions

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Amyloidosis Amyloid Cardiomyopathy Transthyretin Amyloidosis Cardiomyopathies Heart Diseases Polyneuropathies

Keywords

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Amyloidosis ATTR-CM ATTR-PN Transthyretin Amyloid TTR

Study Design

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Allocation Method

RANDOMIZED

Intervention Model

PARALLEL

Primary Study Purpose

PREVENTION

Blinding Strategy

QUADRUPLE

Participants Caregivers Investigators Outcome Assessors

Study Groups

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acoramidis

Participants will receive acoramidis 712 mg orally BID (which is equivalent to 800 mg acoramidis HCl BID)

Group Type EXPERIMENTAL

Acoramidis

Intervention Type DRUG

TTR stabilizer administered orally twice daily (BID)

Placebo

Subjects will receive placebo to match twice daily

Group Type PLACEBO_COMPARATOR

Placebo oral tablet

Intervention Type DRUG

Non-active control administered orally twice daily (BID)

Interventions

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Acoramidis

TTR stabilizer administered orally twice daily (BID)

Intervention Type DRUG

Placebo oral tablet

Non-active control administered orally twice daily (BID)

Intervention Type DRUG

Other Intervention Names

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AG10 ALXN2060

Eligibility Criteria

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Inclusion Criteria

* Male or female ≥ 18 to ≤ 75 years of age inclusive.
* Participants must have an established genotype (hetero- or homozygosity) of a TTR gene variant that is known to be pathogenic (eg, V30M/p.V50M, V122I/p.V142I, T60A/p.T80A, or any other pathogenic TTR variant(s)) confirmed by central laboratory prior to randomization.
* Participant's age is no more than 10 years (≤ 10) younger than the PADO.

Exclusion Criteria

* Evidence of ATTR-CM or ATTR-PN.
* Presence of a TTR variant known to be phenotypically protective (eg, T119M, R104H).
* Current or past treatment with other TTR modifying therapies.
* Contraindication to or inability to undergo Cardiac magnetic resonance testing.
* Major organ dysfunction, including: kidney disease, liver disease, heart disease (including cardiomyopathy), neuropathy
* Other diseases or conditions such has cancer within 3 years, untreated hyperthyroidism or hypothyroidism, type 1 diabetes, active hepatitis B or C, HIV.
* Major surgery within the past 3 months or planned during the next 12 months.
* Known hypersensitivity to acoramidis.
Minimum Eligible Age

18 Years

Maximum Eligible Age

75 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Eidos Therapeutics, a BridgeBio company

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Locations

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University of California, San Diego (UCSD) - Medical Center

La Jolla, California, United States

Site Status RECRUITING

University of California, Los Angeles (UCLA) - David Geffen School of Medicine

Los Angeles, California, United States

Site Status RECRUITING

University of California, San Francisco (UCSF)

San Francisco, California, United States

Site Status RECRUITING

Stanford University

Stanford, California, United States

Site Status RECRUITING

University of Colorado Anschutz

Aurora, Colorado, United States

Site Status RECRUITING

Yale University School of Medicine - Section of Cardiology

New Haven, Connecticut, United States

Site Status RECRUITING

MedStar Washington Hospital Center - MedStar Heart and Vascular Institute

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

Site Status RECRUITING

Mayo Clinic - Jacksonville

Jacksonville, Florida, United States

Site Status RECRUITING

Emory University School of Medicine

Atlanta, Georgia, United States

Site Status RECRUITING

John H. Stroger, Jr. Hospital of Cook County

Chicago, Illinois, United States

Site Status RECRUITING

University of Chicago - Medical Center

Chicago, Illinois, United States

Site Status RECRUITING

University of Maryland Medical Center

Baltimore, Maryland, United States

Site Status RECRUITING

Johns Hopkins University

Baltimore, Maryland, United States

Site Status RECRUITING

Massachusetts General Hospital

Boston, Massachusetts, United States

Site Status RECRUITING

Brigham and Women's Hospital

Boston, Massachusetts, United States

Site Status RECRUITING

Boston University (BU) School of Medicine

Boston, Massachusetts, United States

Site Status RECRUITING

Henry Ford Health System

Detroit, Michigan, United States

Site Status RECRUITING

Mayo Clinic - Rochester

Rochester, Minnesota, United States

Site Status RECRUITING

St. Luke's Hospital of Kansas City

Kansas City, Missouri, United States

Site Status RECRUITING

Washington University in St. Louis

St Louis, Missouri, United States

Site Status RECRUITING

Rutgers-Robert Wood Johnson Medical School

New Brunswick, New Jersey, United States

Site Status RECRUITING

New York University (NYU) School of Medicine - Langone Medical Center

New York, New York, United States

Site Status RECRUITING

Mount Sinai Hospital

New York, New York, United States

Site Status RECRUITING

Columbia University Medical Center

New York, New York, United States

Site Status RECRUITING

Laurelton Heart Specialists

Rosedale, New York, United States

Site Status RECRUITING

Duke University Medical Center

Durham, North Carolina, United States

Site Status RECRUITING

Cleveland Clinic

Cleveland, Ohio, United States

Site Status RECRUITING

Oregon Health & Science University

Portland, Oregon, United States

Site Status RECRUITING

Penn Presbyterian Medical Center

Philadelphia, Pennsylvania, United States

Site Status RECRUITING

University of Pittsburgh Medical Center, Presbyterian Hospital

Pittsburgh, Pennsylvania, United States

Site Status RECRUITING

Medical University of South Carolina (MUSC)

Charleston, South Carolina, United States

Site Status RECRUITING

Prisma Health Cancer Institute

Greenville, South Carolina, United States

Site Status RECRUITING

National Neuromuscular Research Institute

Austin, Texas, United States

Site Status RECRUITING

University of Texas Southwestern

Dallas, Texas, United States

Site Status RECRUITING

University of Utah

Salt Lake City, Utah, United States

Site Status RECRUITING

INOVA Fairfax Hospital

Falls Church, Virginia, United States

Site Status RECRUITING

Virginia Commonwealth University

Richmond, Virginia, United States

Site Status RECRUITING

Hospital Italiano de Buenos Aires

Buenos Aires, , Argentina

Site Status RECRUITING

St Vincent's Hospital Sydney

Darlinghurst, , Australia

Site Status RECRUITING

Westmead Hospital

Westmead, , Australia

Site Status RECRUITING

UZ Leuven

Leuven, , Belgium

Site Status RECRUITING

Fundacao Centro Medico de Campinas

Campinas, , Brazil

Site Status RECRUITING

Instituto do Coracao (InCor) do Hospital das Clinicas da FMUSP

Cerqueira César, , Brazil

Site Status RECRUITING

Cardiopulmonar da Bahia S.A. (Hospital Cardio Pulmonar)

Salvador, , Brazil

Site Status RECRUITING

University of Calgary

Calgary, Alberta, Canada

Site Status RECRUITING

University of British Columbia

Vancouver, British Columbia, Canada

Site Status RECRUITING

Hvitfeldt Poulsen

Aarhus, , Denmark

Site Status RECRUITING

CHU Bordeaux - Hopital Pellegrin

Bordeaux, , France

Site Status RECRUITING

AP-HP Hopital Henri Mondor

Créteil, , France

Site Status RECRUITING

AP-HP Hopital Bicetre

Le Kremlin-Bicêtre, , France

Site Status RECRUITING

CHU Rennes

Rennes, , France

Site Status RECRUITING

CHU de Toulouse - Hopital Rangueil

Toulouse, , France

Site Status RECRUITING

Charite Universitaetsmedizin Berlin

Berlin, , Germany

Site Status RECRUITING

Universitaetsklinikum Heidelberg

Heidelberg, , Germany

Site Status RECRUITING

The Mater Misericordiae University Hospital

Dublin, , Ireland

Site Status RECRUITING

Tallaght University Hospital - The Adelaide and Meath Hospital

Dublin, , Ireland

Site Status RECRUITING

Azienda Ospedaliero-Universitaria di Bologna IRCCS Policlinico di S.Orsola

Bologna, , Italy

Site Status RECRUITING

Azienda Ospedaliero-Universitaria Careggi

Florence, , Italy

Site Status RECRUITING

Fondazione IRCCS Policlinico San Matteo

Pavia, , Italy

Site Status RECRUITING

Fondazione Toscana Gabriele Monasterio per la Ricerca Medica e di Sanità Pubblica - Ospedale San Cataldo

Pisa, , Italy

Site Status RECRUITING

Fondazione Policlinico Universitario A. Gemelli IRCCS - Universita Cattolica del Sacro Cuore

Rome, , Italy

Site Status RECRUITING

Azienda Ospedaliero-Universitaria Sant'Andrea

Rome, , Italy

Site Status RECRUITING

University Malaya Medical Centre (UMMC)

Kuala Lumpur, , Malaysia

Site Status RECRUITING

University Medical Center Groningen

Groningen, , Netherlands

Site Status RECRUITING

Erasmus MC

Rotterdam, , Netherlands

Site Status RECRUITING

Universitair Medisch Centrum Utrecht

Utrecht, , Netherlands

Site Status RECRUITING

Unidade Local de Saude de Santa Maria EPE - Hospital de Santa Maria

Lisbon, , Portugal

Site Status RECRUITING

Unidade Local de Saude de Santo Antonio EPE - Hospital de Santo Antonio

Porto, , Portugal

Site Status RECRUITING

Singapore General Hospital

Singapore, , Singapore

Site Status RECRUITING

Seoul National University Hospital

Seoul, , South Korea

Site Status RECRUITING

Severance Hospital, Yonsei University Health System

Seoul, , South Korea

Site Status RECRUITING

Samsung Medical Center

Seoul, , South Korea

Site Status RECRUITING

Seoul St. Mary's Hospital

Seoul, , South Korea

Site Status RECRUITING

Hospital Universitari de Bellvitge

Barcelona, , Spain

Site Status RECRUITING

Hospital Universitario Juan Ramon Jimenez

Huelva, , Spain

Site Status RECRUITING

Hospital Universitario Puerta de Hierro

Majadahonda, , Spain

Site Status RECRUITING

Hospital Universitario Son Llatzer

Palma de Mallorca, , Spain

Site Status RECRUITING

Hospital Clinico Universitario de Salamanca

Salamanca, , Spain

Site Status RECRUITING

Norrlands universitetssjukhus (University Hospital of Umea)

Umeå, , Sweden

Site Status RECRUITING

Changhua Christian Hospital - Taiwan

Changhua, , Taiwan

Site Status RECRUITING

National Taiwan University Hospital

Taipei, , Taiwan

Site Status RECRUITING

Taipei Veterans General Hospital

Taipei, , Taiwan

Site Status RECRUITING

St. Bartholomew's Hospital

London, , United Kingdom

Site Status RECRUITING

University College London Hospitals NHS Foundation Trust

London, , United Kingdom

Site Status RECRUITING

Countries

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Bulgaria United States Argentina Australia Belgium Brazil Canada Denmark France Germany Ireland Italy Malaysia Netherlands Portugal Singapore South Korea Spain Sweden Taiwan United Kingdom

Central Contacts

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Medical Information

Role: CONTACT

Phone: 1-415-887-1471

Email: [email protected]

Other Identifiers

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U1111-1307-8107

Identifier Type: OTHER

Identifier Source: secondary_id

jRCT2071250116

Identifier Type: OTHER

Identifier Source: secondary_id

AG10-501

Identifier Type: -

Identifier Source: org_study_id