Inge Kühl, invited by Deborah Tribouillard-Tanvier I2BC Institut Biologie Intégrative de la Cellule _ CEA, CNRS, Université Paris-Saclay
Group: Mammalian Mitochondrial Gene Expression and Function in Health and Disease (MATRIX) Dep. Biologie Cellulaire, Institut de Biologie Intégrative de la Cellule, UMR9198
Titre : Novel players in mitochondrial gene expression
Résumé: Mitochondrial dysfunction is related to many pathologies, including certain cancers, and neurodegenerative diseases. Mitochondria are essential organelles that play a key role in various cellular processes including the production of energy in the form of ATP via oxidative phosphorylation (OXPHOS). OXPHOS is coordinately controlled by both, the nuclear genome and the mitochondrial genome (mtDNA). Thus, expression and maintenance of the mtDNA is critical for cellular energy homeostasis. Mitochondria are in essence a prokaryotic system without membrane-based compartmentalization to separate the different steps of the mitochondrial gene expression pathway. Both, transcription and maintenance of the mammalian mtDNA depend on the sole mitochondrial RNA polymerase (POLRMT) that produces primers for mtDNA replication and transcribes the mtDNA in collaboration with a couple transcription factors. Although several factors involved in mitochondrial RNA metabolism have been described, the mechanisms that regulate the link between transcription and translation in mammalian mitochondria is not well understood. We have been studying the regulatory role of human and mouse POLRMT and I will present our novel insights.