Welcome

The main research interest of our lab is on the basic mechanisms that govern the biogenesis of mitochondrial protein complexes in health, disease and aging. We are most specifically interested in the assembly and function of the mitochondrial translation machinery and of the mitochondrial respiratory chain and oxidative phosphorylation system components, involved in biological energy transduction.

The study of mitochondria is now a very “hot issue” for both basic and clinical research. Mitochondria are not only the “energy factories” of the cell but they also house a multiplicity of pathways that serve to regulate cellular life and death. Importantly, mitochondria are involved in prevalent human diseases of wide social impact, most notably neurodegenerative disorders, but also in cancer and the aging process.

In addition, alterations in mitochondrial production of energy are the cause of fatal childhood diseases such as neuromyopathies and cardiomyopathies. Despite more than 50 years of work on the topic, a full understanding of mitochondrial biogenesis at the molecular level has not yet been achieved. The process is complicated by the contribution of two genetic systems, nuclear and mitochondrial, and by the multiple levels of regulation involved, from gene expression to multimeric enzyme assembly and incorporation of metal prosthetic groups. We are committed to the identification of the players involved and the characterization of the mechanisms underlying mitochondrial biogenesis and its regulation. Such information is a prerequisite to the development of therapies to prevent or combat mitochondrion-related disorders. To achieve our goals we are using the facultative aerobe/anaerobe yeast Saccharomyces cerevisiae as a model organism and applying innovative gene-editing approaches to human cultured cells to validate and/or complement the discoveries made in yeast.