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.
Export Options
About this article
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 |
Call for Papers in Thematic Issues
Medicinal Chemistry Advancement in Life-Threatening Diseases
The current issue will highlight concise reports that specify ground-breaking insights, including the novel discovery of drug targets and their action mechanism or drugs of novel classes. These are projected to encourage medicinal chemistry future efforts to address the most challenging medical needs. The current issue highlights further efforts to ...read more
- Author Guidelines
- Graphical Abstracts
- Fabricating and Stating False Information
- Research Misconduct
- Post Publication Discussions and Corrections
- Publishing Ethics and Rectitude
- Increase Visibility of Your Article
- Archiving Policies
- Peer Review Workflow
- Order Your Article Before Print
- Promote Your Article
- Manuscript Transfer Facility
- Editorial Policies
- Allegations from Whistleblowers
- Announcements
Related Articles
-
Genetic and Non-genetic Determinants of Cardiovascular Disease in South Asians
Current Diabetes Reviews Patent Annotations
Recent Patents on Cardiovascular Drug Discovery Oxidative Stress and Endothelial Dysfunction in Cardiovascular Disease: Mitochondria-Targeted Therapeutics
Current Medicinal Chemistry Recent Progress and Related Patents on the Applications of Bone Marrow-Derived Stem/Progenitor Cells in Regenerative Medicine and Cancer Therapies
Recent Patents on Regenerative Medicine High-Density Lipoprotein: Key Molecule in Cholesterol Efflux and the Prevention of Atherosclerosis
Current Pharmaceutical Design Use of Medication in Coronary Imaging by CT
Current Medical Imaging RAGE: A Multi-Ligand Receptor Unveiling Novel Insights in Health and Disease
Current Medicinal Chemistry Hypoxia and Fetal Heart Development
Current Molecular Medicine Preclinical Development of New Therapy for Glycogen Storage Diseases
Current Gene Therapy Transposable Elements in Cancer and Other Human Diseases
Current Cancer Drug Targets Ivabradine: A Current Overview
Current Clinical Pharmacology Metabolomics and Heart Diseases: From Basic to Clinical Approach
Current Medicinal Chemistry Targeting the Chemokines in Cardiac Repair
Current Pharmaceutical Design Patent Selections:
Recent Patents on Endocrine, Metabolic & Immune Drug Discovery (Discontinued) Editorial from Editor-in-Chief [Cheyne-Stokes: A Flashback to the 19th Century]
Current Respiratory Medicine Reviews Markers of Arrhythmogenic Risk in Hypertensive Subjects
Current Pharmaceutical Design Pharmacokinetics of Antifungal Agents in Neonates and Young Infants
Current Medicinal Chemistry Editorial (Thematic Issue: Managing Strategies for Diverse Diseases: Challenges from Bench to Bedside Translation in Successful Drug Discovery and Development (Part B))
Current Pharmaceutical Design Angiotensin Converting Enzyme Inhibitors and Angiotensin II Receptor Antagonists in Experimental Myocarditis
Current Pharmaceutical Design Pharmacologic Therapy in Growth Hormone Disorders and the Heart
Current Medicinal Chemistry