Abstract
Human mitochondrial DNA (mtDNA) codes for 13 polypeptides which constitute the central core of the oxidative phosphorylation (OXPHOS) complexes. The machinery for mitochondrial protein synthesis has a dual origin: a full set of tRNAs, as well as the 12S and 16S rRNAs are encoded in the mitochondrial genome, while most factors necessary for translation are encoded by nuclear genes. The mitochondrial translation apparatus is highly specialized in expressing membrane proteins, and couples the synthesis of proteins to the insertion into the mitochondrial inner membrane. In recent years it has become clear that defects of mitochondrial translation and protein assembly cause several mitochondrial disorders. Since direct studies on protein synthesis in human mitochondria are still a relatively difficult task, we owe our current knowledge of this field to the large amount of genetic and biochemical studies performed in the yeast Saccharomyces cerevisiae. These studies have allowed the identification of several genes involved in mitochondrial protein synthesis and assembly, and have provided insights into the conserved mechanisms of mitochondrial gene expression. In the present review we will discuss the most recent advances in the understanding of the mechanisms and factors that govern mammalian mitochondrial translation/protein insertion, as well as known pathologies associated with them.
Keywords: Assembly, mitochondria, mitochondrial disease, mitochondrial DNA (mtDNA), mitoribosomes, oxidative phosphorylation (OXPHOS), translation, yeast
Current Topics in Medicinal Chemistry
Title: Protein Synthesis and Assembly in Mitochondrial Disorders
Volume: 8 Issue: 15
Author(s): Xochitl Perez-Martinez, Soledad Funes, Yolanda Camacho-Villasana, Sanna Marjavaara, Faviola Tavares-Carreon and Miguel Shingu-Vazquez
Affiliation:
Keywords: Assembly, mitochondria, mitochondrial disease, mitochondrial DNA (mtDNA), mitoribosomes, oxidative phosphorylation (OXPHOS), translation, yeast
Abstract: Human mitochondrial DNA (mtDNA) codes for 13 polypeptides which constitute the central core of the oxidative phosphorylation (OXPHOS) complexes. The machinery for mitochondrial protein synthesis has a dual origin: a full set of tRNAs, as well as the 12S and 16S rRNAs are encoded in the mitochondrial genome, while most factors necessary for translation are encoded by nuclear genes. The mitochondrial translation apparatus is highly specialized in expressing membrane proteins, and couples the synthesis of proteins to the insertion into the mitochondrial inner membrane. In recent years it has become clear that defects of mitochondrial translation and protein assembly cause several mitochondrial disorders. Since direct studies on protein synthesis in human mitochondria are still a relatively difficult task, we owe our current knowledge of this field to the large amount of genetic and biochemical studies performed in the yeast Saccharomyces cerevisiae. These studies have allowed the identification of several genes involved in mitochondrial protein synthesis and assembly, and have provided insights into the conserved mechanisms of mitochondrial gene expression. In the present review we will discuss the most recent advances in the understanding of the mechanisms and factors that govern mammalian mitochondrial translation/protein insertion, as well as known pathologies associated with them.
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Cite this article as:
Perez-Martinez Xochitl, Funes Soledad, Camacho-Villasana Yolanda, Marjavaara Sanna, Tavares-Carreon Faviola and Shingu-Vazquez Miguel, Protein Synthesis and Assembly in Mitochondrial Disorders, Current Topics in Medicinal Chemistry 2008; 8 (15) . https://dx.doi.org/10.2174/156802608786141124
DOI https://dx.doi.org/10.2174/156802608786141124 |
Print ISSN 1568-0266 |
Publisher Name Bentham Science Publisher |
Online ISSN 1873-4294 |
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