Clinical trial • Phase III • Musculoskeletal

HUMANISED IGG1 MONOCLONAL ANTIBODY AGAINST TFR1 CONJUGATED TO DOUBLE STRANDED SIRNA OLIGONUCLEOTIDE AGAINST DMPK VIA A NON-CLEAVABLE LINKER for Myotonic dystrophy type 1

Phase III trial of HUMANISED IGG1 MONOCLONAL ANTIBODY AGAINST TFR1 CONJUGATED TO DOUBLE STRANDED SIRNA OLIGONUCLEOTIDE AGAINST DMPK VIA A NON-CLEAVABLE LI…

Overview

Trial Therapeutic Area
Musculoskeletal
Trial Disease
Myotonic dystrophy type 1
Trial Stage
Phase III
Drug Modality
Monoclonal antibody|Oligonucleotide|ADC
Paediatric Trial
Yes
Orphan Drug
Yes

Key dates

Initial CTIS Submission Date
06-06-2025
First CTIS Authorization Date
29-09-2025

Trial design

open-label Phase III trial across 8 sites in Denmark, France, Germany and others.

Open Label
Yes
Target Sample Size
171

Eligibility

Recruits 171 paediatric patients.

Pregnancy Exclusion
1. Females who are pregnant, breastfeeding, or planning to become pregnant during the study period.
Vulnerable Population
Minors are included: "When enrolling participants who are minors, it is necessary to also obtain consent from a legally authorized representative and the participant will receive information in a way adapted to their age and mental maturity." Assent and parental consent documents are provided (assent, parental ICF files present); subject information and ICFs are available in multiple languages as per national Part II documents.

Inclusion criteria

  • {"criterion_text":"- 1. Ability to provide written informed consent (signed and dated) and any authorizations required by local law and be willing and able to comply with all study requirements. When enrolling participants who are minors, it is necessary to also obtain consent from a legally authorized representative and the participant will receive information in a way adapted to their age and mental maturity.\n- 2. Completion of any prior AOC 1001 studies with satisfactory completion of dosing and follow-up assessments and satisfactory compliance with the protocol requirements of the parent study, as determined by the Investigator."}

Exclusion criteria

  • {"criterion_text":"- 1. Females who are pregnant, breastfeeding, or planning to become pregnant during the study period.\n- 2. Unwilling to comply with contraceptive requirements.\n- 3. Any new conditions or worsening of existing condition that in the opinion of the Investigator would make the participant unsuitable for the study."}

Endpoints

Primary endpoints

  • {"endpoint_text":"- 1. Incidence of TEAEs","definition_or_measurement_approach":""}

Recruitment

Planned Sample Size
171
Recruitment Window Months
57
Consent Approach
Written informed consent is required. For minors, consent must also be obtained from a legally authorized representative and participants receive information adapted to their age and mental maturity. Assent and parental ICF documents are provided. Subject information and informed consent forms are available in multiple languages (English, French, Spanish, Dutch, Italian and country-specific versions as per Part II documents).

Geography

Total Number Of Sites
8
Total Number Of Participants
46

Denmark

Earliest CTIS Part Ii Submission Date
28-08-2025
Latest Decision Or Authorization Date
29-09-2025
Processing Time Days
32
Number Of Sites
2
Number Of Participants
7

Sites

Site Name
Aarhus Universitethospital
Department Name
Neurological department
Contact Person Name
Henning Andersen
Contact Person Email
hennande@rm.dk
Site Name
Copenhagen University Hospital
Department Name
Neuromuskulaer klinik
Contact Person Name
Nicolai Preisler

France

Earliest CTIS Part Ii Submission Date
15-08-2025
Latest Decision Or Authorization Date
02-10-2025
Processing Time Days
48
Number Of Sites
1
Number Of Participants
5

Sites

Site Name
Hopitaux Universitaires Pitie Salpetriere
Department Name
Neuromyologie
Contact Person Name
Guillaume Bassez
Contact Person Email
guillaume.bassez@aphp.fr

Germany

Earliest CTIS Part Ii Submission Date
28-07-2025
Latest Decision Or Authorization Date
06-10-2025
Processing Time Days
70
Number Of Sites
1
Number Of Participants
8

Sites

Site Name
LMU Klinikum Muenchen AöR
Department Name
Department of Neurology
Contact Person Name
Stephan Wenninger

Spain

Earliest CTIS Part Ii Submission Date
20-08-2025
Latest Decision Or Authorization Date
30-09-2025
Processing Time Days
41
Number Of Sites
1
Number Of Participants
3

Sites

Site Name
Hospital Universitario Donostia
Department Name
Neurology
Contact Person Name
Roberto Fernandez Torron

Netherlands

Earliest CTIS Part Ii Submission Date
01-09-2025
Latest Decision Or Authorization Date
01-10-2025
Processing Time Days
30
Number Of Sites
2
Number Of Participants
13

Sites

Site Name
Radboud universitair medisch centrum Stichting
Department Name
Neurology
Contact Person Name
Karlien Mul
Contact Person Email
karlien.mul@radboudumc.nl
Site Name
Academisch Ziekenhuis Maastricht
Department Name
Neurology
Contact Person Name
Catharina Faber
Contact Person Email
c.faber@mumc.nl

Italy

Earliest CTIS Part Ii Submission Date
11-08-2025
Latest Decision Or Authorization Date
02-10-2025
Processing Time Days
52
Number Of Sites
1
Number Of Participants
10

Sites

Site Name
Centro Clinico Nemo
Department Name
Centro Clinico NeMO di Milano
Contact Person Name
Valeria Sansone

Sponsor

Primary sponsor

Full Name
Avidity Biosciences Inc.
Organisation Type
Pharmaceutical company
Country Of Registered Address
United States

Third parties

  • {"country":"United States","full_name":"Medidata Solutions Inc.","duties_or_roles":"code:7","organisation_type":"Non-Pharmaceutical company"}
  • {"country":"United States","full_name":"Trinds LLC","duties_or_roles":"code:15: Clinical Evaluator Oversight & Motor Function Test Training/Activities","organisation_type":"Pharmaceutical company"}
  • {"country":"Finland","full_name":"Medpace Finland Oy","duties_or_roles":"codes:1,11,12,2,4,5,6,7","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Atreo Inc.","duties_or_roles":"code:3","organisation_type":"Non-Pharmaceutical company"}
  • {"country":"United States","full_name":"Gray Consulting Inc.","duties_or_roles":"code:15: Patient Travel and Reimbursement Support","organisation_type":"Pharmaceutical company"}
  • {"country":"United Kingdom","full_name":"Chillibean Limited","duties_or_roles":"code:15: Video Hand Opening Time Assesment","organisation_type":"Pharmaceutical company"}
  • {"country":"Germany","full_name":"ProPharma Group GmbH","duties_or_roles":"code:8","organisation_type":"Pharmaceutical company"}

Investigational products

Investigational Product Name
AOC 1001
Active Substance
HUMANISED IGG1 MONOCLONAL ANTIBODY AGAINST TFR1 CONJUGATED TO DOUBLE STRANDED SIRNA OLIGONUCLEOTIDE AGAINST DMPK VIA A NON-CLEAVABLE LINKER
Modality
Monoclonal antibody|Oligonucleotide|ADC
Routes Of Administration
INTRAVENOUS
Route
INTRAVENOUS
Orphan Designation
Yes
Maximum Dose
4 mg/kg (max daily); max total dose amount 112 (dose units mg/kg as provided)

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