RecruitingNCT04920812

MITOMICS : a Multi-OMICS Approach for the Diagnosis of Mitochondrial Diseases

Interest of Multi-omics (WES / RNA-Seq) Approach to Fight Against the Diagnostic Deadlock in Mitochondrial Diseases


Sponsor

Centre Hospitalier Universitaire de Nice

Enrollment

66 participants

Start Date

Mar 7, 2022

Study Type

OBSERVATIONAL

Conditions

Summary

MITOMICS aims to determine which RNA-Seq results (from muscle or fibroblasts) are the most informative for the interpretation of VUS identified by WES for patients suspected of mitochondrial myopathy. Analysis of RNA-Seq and WES results will performed with a computational approach using an autoencoder-based method


Eligibility

Inclusion Criteria6

  • Patients suspected of a mitochondrial disease with muscular signs (clinical, histological or biochemical)
  • Patients with negative mtDNA and WES NGS in trio
  • Patients with routine muscle and skin biopsies available
  • Blood samples from parents and / or relatives available for segregation studies
  • Informed consent of the study signed by the patient or the legal representatives of the minor patient or under guardianship
  • Patients affiliated to social security

Exclusion Criteria5

  • Patients with suspected mitochondrial disease without muscle involvement
  • Patients for whom the mtDNA NGS and WES have not been performed
  • Patients with suspected mitochondrial disease with causal variant identified
  • Refusal to sign the informed consent for the study
  • Insufficient amount of frozen material or culture failure for fibroblasts

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Interventions

GENETICdiagnosis of mitochondrial myopathy

• Determination of the presence of specific molecular signatures at the RNA level in muscles and fibroblasts from patients


Locations(9)

CHU de Nice

Nice, CHU de NICE, France

C.H.R.U. Brest

Brest, France, France

Chu de Nantes

Nantes, France, France

chu Angers

Angers, France

Chu Brest

Brest, France

APHM

Marseille, France

APHM

Marseille, France

Chu Montpellier

Montpellier, France

CHU Nantes

Nantes, France

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NCT04920812


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