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Animal Models of OXPHOS Disorders
2007Dysfunction of the mitochondrial respiratory chain has been associated with a wide range of human diseases ranging from diabetes to cardiomyopathy. Mutations in a number of nuclear as well as mitochondrial genes have been implicated in causing these diseases.
Nicole Hance, Nils-Göran Larsson
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OXPHOS defects and mitochondrial DNA mutations in cardiomyopathy
Muscle & Nerve, 1995Defects of the mitochondrial respiratory chain in cardiac muscle are an important, yet still overlooked cause of heart failure. In 16 of 32 endocardial biopsies from infants affected by "idiopathic" hypertrophic cardiomyopathy we demonstrated a remarkable decrease of activity of either complex I, or complex IV, or both, relative to complex II + III ...
Zeviani M. +5 more
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2007
In this review the composition, structure and function of the complexes of the mammalian mitochondrial electron transport chain and the ATP synthase are highlighted in the context of the Chemiosmotic Hypothesis and our understanding of oxidative phosphorylation. Thus, a firm biochemical foundation is established for the diagnosis of human mitochondrial
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In this review the composition, structure and function of the complexes of the mammalian mitochondrial electron transport chain and the ATP synthase are highlighted in the context of the Chemiosmotic Hypothesis and our understanding of oxidative phosphorylation. Thus, a firm biochemical foundation is established for the diagnosis of human mitochondrial
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Therapeutic Options in OXPHOS Disorders
2007No curative treatment of OXPHOS disorders is currently available, despite great progress in our understanding of the molecular bases of these diseases. We review available and experimental therapeutic approaches that fall into the following categories:
Rob C. A. Sengers +4 more
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OXPHOS coupling and uncoupling
Coupling of OXPHOS is classically studied by comparing ADP-stimulated O2 flux (OXPHOS capacity P at saturating ADP and Pi concentrations) with non-phosphorylating LEAK respiration L, maintained mainly to compensate for the proton leak at high chemiosmotic potential in mitochondrial preparations supplied with fuel substrates to support mitochondrial ...Daltro Cardoso, Luiza Helena +1 more
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Molecular Biology of the OXPHOS System
2007The mitochondrion contains a circular DNA genome (mtDNA) that serves as the basis for its own genetic system. This system is semiautonomous because the coding capacity of mtDNA is Umited to 13 subunits of the respiratory chain apparatus and the rRNAs and tRNAs necessary for their translation. The inheritance of mtDNA differs from that of nuclear DNA in
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Secondary coenzyme Q10 deficiencies in oxidative phosphorylation (OXPHOS) and non-OXPHOS disorders
2018CoQ deficiency study group: et al.
Yubero, Delia +10 more
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Cell Biological Consequences of OXPHOS Disorders
2007During the past century mitochondria have been recognized to play a central role in many cellular functions. Apart from producing cellular energy in the form of ATP (adenosine 5′-triphosphate) this organelle harbors essential parts of the urea cycle and is crucial for the breakdown of fatty acids, heat generation and the biosynthesis of heme ...
Werner J. H. Koopman +3 more
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Proteolytic coordination of the OXPHOS Life Cycle
Biochemical JournalAbstract The mitochondrial oxidative phosphorylation (OXPHOS) system consists of multimeric, highly ordered protein complexes critical for energy production and metabolic wiring in the cell. Recent discoveries in mitochondrial proteolysis, facilitated by advances in proteomic approaches, have transformed the view of mitochondrial ...
Nataliia Nechytailo +1 more
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