Results 251 to 260 of about 46,956 (278)
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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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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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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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Blue-Native Electrophoresis to Study the OXPHOS Complexes
2020Blue-native polyacrylamide gel electrophoresis (BN-PAGE) is a technique optimized for the analysis of the five components of the mitochondrial oxidative phosphorylation (OXPHOS) system. BN-PAGE is based on the preservation of the interactions between the individual subunits within the integral complexes.
Fernandez-Vizarra, Erika +1 more
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Mitochondrial DNA and OXPHOS Disorders
2007Mitochondrial OXPHOS is at the crosspoint of two quite different genetic systems, the nuclear genome, and the mitochondrial genome (mitochondrial DNA, mtDNA). The latter encodes a few essential components of the mitochondrial respiratory chain, and has unique molecular and genetic properties that account for some of the peculiar features of ...
Massimo Zeviani, Valerio Carelli
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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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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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Biochemical Diagnosis of OXPHOS Disorders
2004In this chapter the biochemical diagnosis of OXPHOS disorders is presented. The laboratory investigations in suspected patients are started with the examination of body fluids. The most important metabolite to be measured is lactate, that is frequendy found to be elevated in blood, urine and cerebrospinal fluid of patients with OXPHOS disorders.
J. M. Frans Trijbels +4 more
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Modulation of oxidative phosphorylation (OXPHOS) by radiation- induced biophotons
Environmental Research, 2018Radiation-induced biophotons are an electromagnetic form of bystander signalling. In human cells, biophoton signalling is capable of eliciting effects in non-irradiated bystander cells. However, the mechanisms by which the biophotons interact and act upon the bystander cells are not clearly understood.
Michelle, Le +7 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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