High energy expenditure is reported in cystic fibrosis (CF) animal models and patients. Alterations in skeletal muscle oxidative capacity, fuel utilization, and the creatine kinase-phosphocreatine system suggest mitochondria' dysfunction. Studies were performed on congenic C57BL/6J and F508del (Cftr(tm1kth)) mice. Indirect calorimetry was used to measure gas exchange to evaluate aerobic capacity during treadmill exercise. The bioenergetic function of skeletal muscle subsarcolemmal (SSM) and interfibrillar mitochondria (IFM) was evaluated using an integrated approach combining measurement of the rate of oxidative phosphorylation by polarography and of electron transport chain activities by spectrophotometry. CF mice have reduced maximal aero...
Aim: To assess changes of mitochondrial function and activities of enzymes involved in the transport...
AbstractWe have blocked creatine kinase (CK)-mediated phosphocreatine (PCr) ⇃ ATP transphosphorylati...
Introduction To study the mechanisms of Wolfram syndrome, we assessed the changes in activities, a...
High energy expenditure is reported in cystic fibrosis (CF) animal models and patients. Alterations ...
International audienceMitochondrial diseases are genetic disorders leading to an impaired mitochondr...
Peripheral muscle dysfunction is an important systemic consequence of cystic fibrosis (CF) with majo...
We have generated an animal model for mitochondrial myopathy by disrupting the gene for mitochondria...
Skeletal muscles respond with high plasticity to pathobiological conditions or changes in physiologi...
Skeletal muscle dysfunction is a common complication and an important prognostic factor in patients ...
Muscle dysfunction and muscle wasting are major systemic manifestations of chronic conditions such a...
AbstractCystic Fibrosis (CF) is a frequent and lethal autosomal recessive disease, caused by mutatio...
Cystic Fibrosis (CF) is a frequent and lethal autosomal recessive disease, caused by mutations in th...
Metabolic dysfunction in skeletal muscle contributes to the aetiology and development of muscle dise...
Contains fulltext : 153971.pdf (publisher's version ) (Closed access)Mitochondrial...
Considerable variation in energy expenditure is observed in C57B1/6 mice on a high-fat diet. Because...
Aim: To assess changes of mitochondrial function and activities of enzymes involved in the transport...
AbstractWe have blocked creatine kinase (CK)-mediated phosphocreatine (PCr) ⇃ ATP transphosphorylati...
Introduction To study the mechanisms of Wolfram syndrome, we assessed the changes in activities, a...
High energy expenditure is reported in cystic fibrosis (CF) animal models and patients. Alterations ...
International audienceMitochondrial diseases are genetic disorders leading to an impaired mitochondr...
Peripheral muscle dysfunction is an important systemic consequence of cystic fibrosis (CF) with majo...
We have generated an animal model for mitochondrial myopathy by disrupting the gene for mitochondria...
Skeletal muscles respond with high plasticity to pathobiological conditions or changes in physiologi...
Skeletal muscle dysfunction is a common complication and an important prognostic factor in patients ...
Muscle dysfunction and muscle wasting are major systemic manifestations of chronic conditions such a...
AbstractCystic Fibrosis (CF) is a frequent and lethal autosomal recessive disease, caused by mutatio...
Cystic Fibrosis (CF) is a frequent and lethal autosomal recessive disease, caused by mutations in th...
Metabolic dysfunction in skeletal muscle contributes to the aetiology and development of muscle dise...
Contains fulltext : 153971.pdf (publisher's version ) (Closed access)Mitochondrial...
Considerable variation in energy expenditure is observed in C57B1/6 mice on a high-fat diet. Because...
Aim: To assess changes of mitochondrial function and activities of enzymes involved in the transport...
AbstractWe have blocked creatine kinase (CK)-mediated phosphocreatine (PCr) ⇃ ATP transphosphorylati...
Introduction To study the mechanisms of Wolfram syndrome, we assessed the changes in activities, a...