Genotype/Phenotype Correlation of MORC2 Mutations

NCT ID: NCT07038239

Last Updated: 2025-07-04

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

Total Enrollment

45 participants

Study Classification

OBSERVATIONAL

Study Start Date

2025-09-30

Study Completion Date

2027-09-30

Brief Summary

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The Microrchidia CW-type zinc finger 2 (MORC2) gene encodes a protein expressed in all tissues and enriched in the brain. It is involved in Charcot-Marie-Tooth disease, with mire than 30 families presenting MORC2 mutations. Recently, MORC2 mutation have been shown to be responsible for more complex phenotypes like DIFGAN: developmental delay, impaired growth, dysmorphic facies and axonal neuropathy.

Different mutations are responsible from a diverse spectrum of phenotype, from CMT to DIFGAN.

MORC2 is involved, through its ATPase activity, in DNA repair, chromatin remodeling and epigenetic silencing via the Human silencing hub (HUSH) complex. Our hypothesis is that the hypo- or hyper-activation of the HUSH complex by different MORC2 mutations could be responsible for different phenotypes in patients. The aim of this study is to perform a genotype-phenotype correlation study in patients presenting MORC2 mutations.

Detailed Description

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Conditions

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Charcot Marie Tooth Disease DIFGAN Developmental Delay (Disorder) Impaired Growth Dysmorphic Facies and Axonal Neuropathy

Study Design

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Observational Model Type

OTHER

Study Time Perspective

PROSPECTIVE

Study Groups

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Charcot-Marie-Tooth patients : Patients presenting with length-dependent sensitive-motor axonal neur

Patients presenting with length-dependent sensitive-motor axonal neuropathy

Skin biopsy

Intervention Type DIAGNOSTIC_TEST

under the arm using a 3 mm punch, with local anaesthesia, in the investigating centres.

Blood sample

Intervention Type DIAGNOSTIC_TEST

3 classical 4ml tubes samples per patients, using the routine blood sampling technique, in the investigating centres.

For children, blood sampling volume will be adapted to the patient's weight according to L.1121-1 of the French public health code.

DIFGAN patients

Patients presenting with DIFGAN syndrome : developmental delay, impaired growth, dysmorphic facies and axonal neuropathy

Skin biopsy

Intervention Type DIAGNOSTIC_TEST

under the arm using a 3 mm punch, with local anaesthesia, in the investigating centres.

Blood sample

Intervention Type DIAGNOSTIC_TEST

3 classical 4ml tubes samples per patients, using the routine blood sampling technique, in the investigating centres.

For children, blood sampling volume will be adapted to the patient's weight according to L.1121-1 of the French public health code.

Control

Control group without any neurological disorder

No interventions assigned to this group

Interventions

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Skin biopsy

under the arm using a 3 mm punch, with local anaesthesia, in the investigating centres.

Intervention Type DIAGNOSTIC_TEST

Blood sample

3 classical 4ml tubes samples per patients, using the routine blood sampling technique, in the investigating centres.

For children, blood sampling volume will be adapted to the patient's weight according to L.1121-1 of the French public health code.

Intervention Type DIAGNOSTIC_TEST

Eligibility Criteria

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Inclusion Criteria

* Presence of a mutation in the MORC2 gene, identified during an evaluation for peripheral neuropathy or intellectual disability
* Patient has undergone electromyography (EMG) or is able to undergo EMG during the inclusion visit
* Affiliation with the national health insurance system
* Informed consent from the patient if an adult, or from parents/legal guardians if the patient is a minor

Exclusion Criteria

* Presence of another mutation responsible for peripheral neuropathy or intellectual disability
* Refusal to undergo biological sample collection
* Pregnant, postpartum, or breastfeeding women
* Individuals deprived of liberty by judicial or administrative decision
* Individuals not affiliated with a social security system or not benefiting from an equivalent health coverage scheme
Minimum Eligible Age

4 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Hospices Civils de Lyon

OTHER

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Locations

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CHU de Besançon

Besançon, , France

Site Status RECRUITING

CHRU Brest

Brest, , France

Site Status RECRUITING

CHU Grenoble

Grenoble, , France

Site Status RECRUITING

CH de Versailles

Le Chesnay, , France

Site Status RECRUITING

Service de Génétique moléculaire, pharmacogénétique, hormologie Hôpital Bicêtre

Le Kremlin-Bicêtre, , France

Site Status RECRUITING

Hospices Civils de Lyon

Lyon, , France

Site Status RECRUITING

CHU Marseille

Marseille, , France

Site Status RECRUITING

CHU de Nantes

Nantes, , France

Site Status RECRUITING

CH Pitié Salpêtrière

Paris, , France

Site Status RECRUITING

Hôpital Necker

Paris, , France

Site Status RECRUITING

CHU de Saint-Etienne

Saint-Etienne, , France

Site Status RECRUITING

CHU Strasbourg

Strasbourg, , France

Site Status RECRUITING

Countries

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France

Central Contacts

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Shams RIBAULT, MD

Role: CONTACT

00334 72 07 25 73

Facility Contacts

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Elise Boucher Brischoux, MD

Role: primary

Audebert Bellanger, MD

Role: primary

Martial Mallaret, MD

Role: primary

Geoffroy Delplancq, MD

Role: primary

Andoni ECHANIZ-LAGUNA, MD

Role: primary

0033145212121

Shams RIBAULT, MD

Role: primary

00334 72 07 25 73

Nathalie BONELLO, MD

Role: primary

Marie Vincent, MD

Role: primary

00332 40 08 32 45

Tanya Stojkovic, MD

Role: primary

Cyril Gitiaux, MD

Role: primary

00331 49 57 43 44

Jean-Philippe Camdessanche, MD

Role: primary

0033477127805

Aleksandra NADAJ PAKLEZA

Role: primary

Other Identifiers

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2025-A00225-44

Identifier Type: OTHER

Identifier Source: secondary_id

69HCL23_0647

Identifier Type: -

Identifier Source: org_study_id

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