معرفی
Metodi Metodiev is a permanent researcher (Chargé de Recherche) at INSERM, based at the Imagine Institute of Université Paris Cité. He leads research in mitochondrial diseases, focusing on impaired mitochondrial translation and proteostasis mechanisms. His work bridges fundamental molecular biology with clinical applications in rare genetic disorders.
His academic journey includes a PhD from the University of Cologne (Germany) studying mitochondrial proteolysis, followed by postdoctoral research at Karolinska Institute (Sweden) and Max Planck Institute for the Biology of Aging (Germany) using knockout mouse models. He obtained his Habilitation à Diriger des Recherches (HDR) from Paris-Descartes University in 2015.
Dr. Metodiev's research centers on molecular mechanisms of mitochondrial diseases, with emphasis on protein processing defects and therapeutic development. His team investigates Leigh syndrome pathogenesis and gene therapy approaches, collaborating closely with Dr. Benedetta Ruzzenente. Current projects explore mitochondrial ribosomal biogenesis and proteostasis networks using patient-derived cellular models.
His 2017-2020 publications reveal consistent focus on genetic mutations disrupting mitochondrial translation machinery (e.g., MRPS28, MRPS34, TRMT10C), linking ribosomal defects to multisystem disorders including sensorineural hearing loss, cardiomyopathy, and neurodegeneration. Research integrates mouse models with human fibroblast studies to identify pathogenic mechanisms and therapeutic targets.
Scientific recognition includes:
- AFM-TELETHON postdoctoral fellowship
Dr. Metodiev supervises postdoctoral researchers including Dr. Martin Horak on mitochondrial protein processing projects. He serves as academic editor for PLoS One and Scientific Reports, contributing to peer review in genetics and molecular medicine. His collaborative network spans multiple Imagine Institute teams working on mitochondrial disease mechanisms and therapies.
As core member of the "Genetics of Mitochondrial Diseases" team at Imagine Institute, he contributes to seven specialized research groups covering mitochondrial RNA maturation, disease-interferon interactions, metabolic flux analysis, gene therapy development, embryonic mtDNA dynamics, Friedreich's ataxia mechanisms, genomic discovery, and the national Reference Center for Mitochondrial Diseases (CARAMMEL).
