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Issues
July 2018
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Cover Image
Cover Image
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).
ISSN 0071-1365
EISSN 1744-1358
In this Issue
Mitochondrial Diseases
Review Articles
Clinical syndromes associated with mtDNA mutations: where we stand after 30 years
Essays Biochem (2018) 62 (3): 235–254.
Assembly of mammalian oxidative phosphorylation complexes I–V and supercomplexes
Essays Biochem (2018) 62 (3): 255–270.
Coenzyme Q10 deficiencies: pathways in yeast and humans
Agape M. Awad; Michelle C. Bradley; Lucía Fernández-del-Río; Anish Nag; Hui S. Tsui; Catherine F. Clarke
Essays Biochem (2018) 62 (3): 361–376.
Advancing genomic approaches to the molecular diagnosis of mitochondrial disease
Essays Biochem (2018) 62 (3): 399–408.
Advances in methods for reducing mitochondrial DNA disease by replacing or manipulating the mitochondrial genome
Essays Biochem (2018) 62 (3): 455–465.