Mitochondrial OXPHOS System: Emerging Concepts and Technologies and Role in Disease

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Background

Mitochondria are the central organelles in cellular metabolism. They house the machinery required for oxidative phosphorylation (OXPHOS), the energy transducing process involving five multi-subunit complexes (Complex I-V) that produce ATP from a series of sequential electron transfer reactions coupled to proton translocation across the mitochondrial inner membrane. Not only do mitochondria provide sufficient energy to perform all cellular tasks, but they are also key for the regulation of many important cellular processes. Many components are necessary for OXPHOS biogenesis, starting from the organelles own genome, the mtDNA, which encodes for 13 polypeptides (all core subunits of the OXPHOS complexes), as well as 22 tRNAs and 2 rRNAs, required for the translation of these 13 genes. The rest of the mitochondrial proteome (approx. 99%), including all the replication, transcription and the rest of the translation and assembly factors are encoded in the nucleus, synthesized in the cytosol, and imported into the mitochondria. Once imported, nuclear-encoded components of the OXPHOS complexes are assembled with their mitochondrial-encoded counterparts in a highly coordinated manner. Various internal mitochondrial quality control processes assist with this coordination by ensuring that proteostasis is maintained and that import levels are regulated.

Mutations in mitochondrial genes, either encoded in the nucleus or in the mtDNA, have been associated with pathological conditions in humans. These severe pathologies, known as mitochondrial diseases, affect different tissues and organs. The underlying molecular pathogenetic mechanisms of these disorders are far from being completely understood and clinically are still difficult to diagnose. Moreover, mitochondrial genetic variation and dysfunction has been shown to influence common diseases, such as cancer, Alzheimer’s, Parkinson’s disease, diabetes, and cardiovascular diseases, among others. Finally, the function of OXPHOS is not only critical to maintain cellular homeostasis, but also to support differentiation processes during embryonic development. The metabolic routes hosted by the organelles and their composition in fact vary with the differentiation into distinct cell types and during different stages of development. This directly impacts the OXPHOS functionality and is most relevant to understand the impact of its dysfunction in a tissue-specific manner.

This Research Topic aims to provide state of the art insights into processes related to OXPHOS biogenesis and function, with specific focus on the intricate processes involved in the synthesis and assembly of the five multimeric complexes that compose the OXPHOS core, and how defects in these processes are linked to disease in humans. Research in these aspects of mitochondrial physiology and pathology is nowadays a very active field that has taken advantage of the development of innovative technologies (e.g high-throughput -omic technologies such as next-generation sequencing and proteomic mass spectrometry), experimental approaches, and different in vitro (stem cells, organoids) and in vivo experimental models (from insects to large animals). Thus, a particular focus of this Research Topic is to update the current knowledge but also to point out the limitations faced by the researchers in the field of mitochondrial patho-physiology by highlighting emerging concepts and technologies to facilitate the diagnosis and treatment of the disease, as well as the tools and model systems that can aid in our fundamental understanding of mitochondrial OXPHOS biogenesis and its role in health and disease. Other aspects such as the role of transporters, and signaling pathways related to mitochondrial biogenesis are not in the focus of this collection.


Areas related to mitochondrial OXPHOS biogenesis and function to be covered in this Research Topic may include:

- mtDNA replication and maintenance
- Mitochondrial gene expression (transcription and translation)
- Protein import into mitochondria
- Mitochondrial dysfunction in complex diseases
- OXPHOS complex assembly: ETC (complexes I-IV) and ATP synthase (complex V) assembly
- OXPHOS and cell differentiation
- Quality control of mitochondrial proteostasis
- Emerging technologies for diagnosing, treating and understanding of mitochondrial disease

Keywords: mitochondrial dysfunction, mitochondrial disease, OXPHOS biogenesis

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