Identification of Novel Biomarkers in Early Charcot-Marie-Tooth 1A Disease

NCT ID: NCT07049588

Last Updated: 2025-07-03

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

NA

Total Enrollment

55 participants

Study Classification

INTERVENTIONAL

Study Start Date

2025-06-24

Study Completion Date

2028-06-24

Brief Summary

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This is a 2-year follow-up study of a cohort of 35 CMT1A patients and 20 healthy volunteers. The main objective is identifying prognostic markers for CMT1A using multi-omics analysis. The study is recruiting subjects between the ages of 10 and 30.

The most common inherited neuropathy is Charcot-Marie-Tooth disease type 1A (CMT1A), caused by a duplication of the gene expressing PMP22. CMT1A patients develop symptoms in early childhood with variable progression and there is no established therapy until now. Therapy must start in childhood, before peripheral nerves degenerate. However, we lack easily obtainable biomarkers in early disease stages.

In CMT-MODs, we will identify disease and prognostic biomarkers in young CMT1A patients.

Detailed Description

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The CMT-MODs project aims to conduct a multi-omics analysis (transcriptomics, proteomics, lipidomics) in young patients with early-stage CMT1A. This evaluation should enable the identification of prognostic and change-sensitive biomarkers for use in clinical trials.

A large cohort of CMT1A children, adolescents and young adults aged 10-30 years over 12 months applying the novel clinical outcome measures CMT Examination Score/CMT Neuropathy Score Version Version 2 Rasch versions (CMTES-R/CMTNSv2-R), the functional outcome measure CMT-FOM, pCMT-Qol, as well as a nerve conduction study (NCS) and quantitative MRI will be assessed.

Blood (and optional skin) samples will be taken and gene expression of the most promising candidates will be identified.

This assessment of CMT patients at early disease stages will allow CMT-MODs to establish biomarkers that may serve as a standard readout for disease severity and predict the disease course.

Conditions

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Charcot-Marie-Tooth Disease Type 1A

Study Design

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Allocation Method

NON_RANDOMIZED

Intervention Model

PARALLEL

Comparison of patient and healthy volunteers data at inclusion (M0) and 12 months (M12)
Primary Study Purpose

OTHER

Blinding Strategy

NONE

Study Groups

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Charcot-Marie-Tooth Neuropathy 1A

Patient with genetically confirmed CMT1A or with a parent whose diagnosis is genetically confirmed,

Group Type OTHER

Quantitative neuromuscular MRI

Intervention Type OTHER

Quantification of biomarkers as fat fraction, magnetization Transfer Ratio, muscular volume, relaxation time T2

Skin biopsy

Intervention Type OTHER

Performed on the arm or index finger, depending on patient age

Clinical scores

Intervention Type OTHER

ONLS, CMTES-R, CMT-Peds, CMT-FOM

Blood test

Intervention Type OTHER

10 ml sample

Patient Report Outcomes Measures

Intervention Type OTHER

pCMT-QoL, EVA, WALK-12, PGI-c, SF-12

Healthy volunteers

Patient-matched controls

Group Type OTHER

Quantitative neuromuscular MRI

Intervention Type OTHER

Quantification of biomarkers as fat fraction, magnetization Transfer Ratio, muscular volume, relaxation time T2

Skin biopsy

Intervention Type OTHER

Performed on the arm or index finger, depending on patient age

Clinical scores

Intervention Type OTHER

ONLS, CMTES-R, CMT-Peds, CMT-FOM

Blood test

Intervention Type OTHER

10 ml sample

Interventions

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Quantitative neuromuscular MRI

Quantification of biomarkers as fat fraction, magnetization Transfer Ratio, muscular volume, relaxation time T2

Intervention Type OTHER

Skin biopsy

Performed on the arm or index finger, depending on patient age

Intervention Type OTHER

Clinical scores

ONLS, CMTES-R, CMT-Peds, CMT-FOM

Intervention Type OTHER

Blood test

10 ml sample

Intervention Type OTHER

Patient Report Outcomes Measures

pCMT-QoL, EVA, WALK-12, PGI-c, SF-12

Intervention Type OTHER

Eligibility Criteria

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Inclusion Criteria

* Healthy volunteer or patient who has given consent for participation in the study or, for minors, a healthy volunteer whose two parents have given consent for participation in the study.
* Patient with genetically confirmed CMT1A or with a parent whose diagnosis is genetically confirmed
* Patient able to walk with or without assistance

Exclusion Criteria

* Healthy volunteer with neurological disorders
* Healthy volunteer or patient with a contraindication to MRI,
* Healthy volunteers or patient under 30 kg
* Helathy volunteer on long-term therapy
* Patient with other neuromuscular pathologies
* Patient in a period of exclusion from another research protocol at the time of signing the consent/non-opposition form
* Pregnant or breast-feeding women
* Subjects covered by articles L1121-5 to 1121-8 of the French Public Health Code (minors, adults under guardianship or trusteeship, patients deprived of their liberty, pregnant or breast-feeding women)
* Subjects who cannot read and understand the French language well enough to be able to give their consent to participate in research
Minimum Eligible Age

10 Years

Maximum Eligible Age

30 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

Yes

Sponsors

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Association Française contre les Myopathies (AFM), Paris

OTHER

Sponsor Role collaborator

University Medical Center Göttingen

UNKNOWN

Sponsor Role collaborator

Assistance Publique Hopitaux De Marseille

OTHER

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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François CREMIEUX

Role: STUDY_DIRECTOR

Assistance Publique - Hôpitaux de Marseille

Locations

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Assistance Publique - Hôpitaux de Marseille

Marseille, , France

Site Status RECRUITING

Countries

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France

Central Contacts

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Shahram ATTARIAN, PU-PH

Role: CONTACT

04 91 38 65 79 ext. +33

Etienne FORTANIER, MD

Role: CONTACT

04 91 38 65 79 ext. +33

Facility Contacts

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François CREMIEUX, Managing Director

Role: primary

04 91 38 27 47 ext. +33

Other Identifiers

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2024-A02403-44

Identifier Type: OTHER

Identifier Source: secondary_id

RCAPHM24_0381

Identifier Type: -

Identifier Source: org_study_id

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