Mitochondria: From Physiology to Pathology

Dear Colleagues

Mitochondria play an increasingly central role in the context of cellular physiology. These organelles possess their own DNA (mtDNA), functionally coordinated with nuclear genome; mitochondrial gene expression is mediated by molecular processes (replication, transcription, translation, and assembly of respiratory chain complexes) that all take place within the mitochondria. Several aspects of mtDNA expression have already been well-characterized, but many more either are still under debate or have yet to be discovered.

Understanding the molecular processes occurring in mitochondria also has a clinical relevance. Dysfunctions affecting these important metabolic ‘hubs’ are associated to a whole range of severe disorders, best known as mitochondrial diseases. In recent years, significant progress has been made to understand the pathogenic mechanisms underlying mitochondrial dysfunction, also thanks to the rapid development of next-generation sequencing technologies. To date, mitochondrial diseases are complex genetic disorders without any effective therapy. Current therapeutic strategies and clinical trials are aimed at mitigating clinical manifestations and slowing the disease progression in order to improve the lifestyle of patients.

The goal of this Special Issue is to collect research and review articles covering the physiological and pathological aspects related to mtDNA maintenance and gene expression, mitochondrial biogenesis and protein import, organelle metabolism and quality control, as well as mitochondrial diseases.

Prof. Francesco Bruni
Guest Editor

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