Mitochondrial disorders can affect apparently unrelated organs, usually before age 1 year, although isolated organ involvement is also seen myocardiopathy, liver failure, etc. The clinical variability of mitochondrial disorders is due to the ubiquitous nature of the mitochondrial respiratory chain. Mitochondrial disorders are studied at the metabolic, biochemical and genetic levels. Metabolic investigations can assist with the diagnosis, especially when they reveal lactic acidosis although the absence of lactic acidosis does not rule out the diagnosis. Enzymatic and molecular investigations are required to confirm the diagnosis, especially through enzyme studies of the different complexes of the respiratory chain in isolated mitochondria or affected tissues. The different proteins of the mitochondrial respiratory chain being encoded partly by the mitochondrial genome and partly by nuclear genes, abnormal oxidative phosphorylation can originate either in the mitochondria or in the nucleus.
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Reactive oxygen species ROS are normal byproducts of mitochondrial respiratory chain activity. We also compared amino acid sequences to try to elucidate the leukemia pathogenesis, in relationship with the mitochondrial respiratory chain and apoptosis.
The methods comprise administering to a mammal a composition containing pyrimidine nucleotide precursors in amounts sufficient to treat symptoms resulting from mitochondrial respiratory chain deficiencies. These changes may be secondary to an underlying genetic defect or may indicate a deficiency of the mitochondrial respiratory chain that predisposes this breed to heart failure.
SdhCDAB is a good model system for the succinate dehydrogenase Sdh found in the mitochondrial respiratory chain complex II , as the subunits are structural homologues. Catena contains the medicinal ingredient idebenone, which is a synthetic short-chain analogue of CoenzymeQ10, a component of mitochondrial respiratory chain. Similarly, an artificial hypoglycemia in vitro glucose consumption is possible if the sampling tube does not contain sodium fluoride this blocks the mitochondrial respiratory chain , so aerobic glycolysis.
MALDI imaging was used to detect defects in the mitochondrial respiratory chain , causing the difference in treatment response for advanced adenocarcinoma of the esophagus. Complex I defects are one of the most frequent causes of diseases and disorders associated with mitochondrial respiratory chain disorders, including genetically related diseases and late onset neurodegenerative diseases.
Another mechanism involves effects on the mitochondrial respiratory enzyme chain that is responsible for effective tissue utilization of oxygen. Kepone had almost no effect, but the few changes that were recorded increased mitochondrial volume in one cell type are consistent with known actions of Kepone alteration of mitochondrial respiration. Protons can be physically moved across a membrane; this is seen in mitochondrial Complexes I and IV.
La Phosphorylation Oxydative Mitochondriale
Traduction de "chaîne respiratoire mitochondriale" en anglais