References in periodicals archive ?
Clinically, the most important application of CoQ10 assay is detection of CoQ10 deficiency of the respiratory chain.
Magnetic resonance spectroscopy is a new neuroimaging technique that can reveal abnormal changes in some metabolic products related to respiratory chain dysfunction by shift of chemical peaks.
In summary, the present study demonstrates that 3NP inhibits the respiratory chain activity in isolated mitochondrial preparation from the striatum and cerebellum of both young and old animals.
Time-dependent and tissue-specific accumulation of mtDNA and respiratory chain defects in chronic doxorubicin cardiomyopathy.
The utility of EPI-743 is also being explored in other inherited respiratory chain disorders.
Edison looks to develop the Phase IB drug candidate EPI-589 for adult central nervous system disease as well as EPI-743, an FDA-designated orphan drug in Phase II clinical development for inherited respiratory chain disorders, with ongoing placebo trials for Leigh syndrome, cobalamin C defect, undiagnosed disorders of oxidation-reduction, and Friedreich's ataxia.
One aspect of mitochondria is the generation of reactive oxygen species, or ROS, the main source in the body from oxygen utilization by respiratory chain reactions.
announced today the United States Food and Drug Administration has granted orphan drug designation to EPI-743 for treatment of inherited mitochondrial respiratory chain diseases.
Some practical aspects of providing a diagnostic service for respiratory chain defects.
Her team continues to work to explore the many different consequences of mitochondrial respiratory chain dysfunction in animal models, and ways in which these consequences might themselves be treated.
The results showed that SMND-309 decreased neurological deficit scores, reduced the number of dead hippocampal neuronal cells in accordance with its depression on mitochondria swelling degree, reactive oxygen species production, improvements on mitochondria swelling, energy metabolism, membrane potential level and mitochondrial respiratory chain complex activities.