Search results for "mitochondrial dysfunction"
showing 4 items of 14 documents
From clinical description, to in vitro and animal studies, and backward to patients: Oxidative stress and mitochondrial dysfunction in Fanconi anemia
2013
Fanconi anemia (FA) is a rare genetic disease associated with deficiencies in DNA repair pathways. A body of literature points to a pro-oxidant state in FA patients, along with evidence for oxidative stress (OS) in the FA phenotype reported by in vitro, molecular, and animal studies. A highlight arises from the detection of mitochondrial dysfunction (MDF) in FA cell lines of complementation groups A, C, D2, and G. As yet lacking, in vivo studies should focus on FA-associated MDF, which may help in the understanding of the mitochondrial basis of OS detected in cells and body fluids from FA patients. Beyond the in vitro and animal databases, the available analytical devices may prompt the dir…
Current Experience in Testing Mitochondrial Nutrients in Disorders Featuring Oxidative Stress and Mitochondrial Dysfunction: Rational Design of Chemo…
2014
An extensive number of pathologies are associated with mitochondrial dysfunction (MDF) and oxidative stress (OS). Thus, mitochondrial cofactors termed "mitochondrial nutrients" (MN), such as alpha-lipoic acid (ALA), Coenzyme Q10 (CoQ10), and L-carnitine (CARN) (or its derivatives) have been tested in a number of clinical trials, and this review is focused on the use of MN-based clinical trials. The papers reporting on MN-based clinical trials were retrieved in MedLine up to July 2014, and evaluated for the following endpoints: (a) treated diseases; (b) dosages, number of enrolled patients and duration of treatment; (c) trial success for each MN or MN combinations as reported by authors. The…
Mitochondrial dysfunction and host immune response during pneumococcal pneumonia : impact of mechanical ventilation?
2020
Lung damage induced by mechanical ventilation (MV) worsens the prognosis of pneumonia. Mitochondrial dysfunctions could account for such deleterious effects. Accordingly, data obtained from both in vitro and in vivo experimental studies, as well as in ventilated patients with acute respiratory distress syndrome, show that mitochondrial alarmins, especially mitochondrial DNA, are released in the alveolar compartment as a result of the cyclic stretch, and could thus represent one link between the mechanical insult and the sterile inflammation leading to VILI (ventilator-induced lung injury). In rabbits, we show herein that adverse and prolonged MV worsens the prognosis of pneumococcal pneumon…
Mitochondrial dysfunction in cholestatic liver diseases
2011
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