Review Articles
Mitochondria are unique organelles under the dual genetic control executed by nuclear DNA and their own genome, mitochondrial DNA. Every cell contains a population of mitochondria and multiple copies of mtDNA, carrying wild type or mutated variants (heteroplasmy). The genetic variability together with the complex regulation of multiple metabolic pathways operating in mitochondria are responsible for phenotypic variability, schematically represented in the cover image. In this issue of Essays in Biochemistry, we illustrate the biological pathways operating in mitochondria and the pathomechanisms leading to disease. We also provide an overview of the current advances in the approach to diagnosis, design of new therapies, and development of clinical trials. Image kindly provided by Caterina Garone (MRC Mitochondrial Biology Unit).
