Evidence for caspase-dependent programmed cell death along with repair processes in affected skeletal muscle fibres in patients with mitochondrial disorders

2015 ◽  
Vol 130 (3) ◽  
pp. 167-181 ◽  
Author(s):  
Valeria Guglielmi ◽  
Gaetano Vattemi ◽  
Roberto Chignola ◽  
Anna Chiarini ◽  
Matteo Marini ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Cell Death ◽  
Programmed Cell Death ◽  
Respiratory Chain ◽  
Mitochondrial Respiratory Chain ◽  
Mitochondrial Disorders ◽  
Muscle Fibres ◽  
Repair Processes ◽  
Skeletal Muscle Fibres ◽  
Mitochondrial Respiratory

We report the role and the relevance of programmed cell death (PCD) in patients with mitochondrial respiratory chain dysfunction. Our data throw light on to the pathogenesis of mitochondrial disorders and notably on to the fate of the affected muscle fibres

Mitochondrial respiratory chain function in skeletal muscle of ALS patients

2002 ◽  
Vol 52 (5) ◽  
pp. 623-627 ◽  
Author(s):  
Andoni Echaniz-Laguna ◽  
Joffrey Zoll ◽  
Florence Ribera ◽  
Christine Tranchant ◽  
Jean-Marie Warter ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Respiratory Chain ◽  
Mitochondrial Respiratory Chain ◽  
Als Patients ◽  
Mitochondrial Respiratory

Biochemical evaluation of mitochondrial respiratory chain enzymes in canine skeletal muscle

2004 ◽  
Vol 65 (4) ◽  
pp. 480-484 ◽  
Author(s):  
Joseph J. Wakshlag ◽  
Barry J. Cooper ◽  
Robin R. Wakshlag ◽  
Francis. A. Kallfelz ◽  
Stephen C. Barr ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Respiratory Chain ◽  
Mitochondrial Respiratory Chain ◽  
Biochemical Evaluation ◽  
Mitochondrial Respiratory

Effects of prolonged fasting on AMPK signaling, gene expression, and mitochondrial respiratory chain content in skeletal muscle from lean and obese individuals

2013 ◽  
Vol 304 (9) ◽  
pp. E1012-E1021 ◽  
Author(s):  
Marjolein A. Wijngaarden ◽  
Gerard C. van der Zon ◽  
Ko Willems van Dijk ◽  
Hanno Pijl ◽  
Bruno Guigas
Keyword(s):  
Gene Expression ◽  
Skeletal Muscle ◽  
Respiratory Chain ◽  
Mitochondrial Respiratory Chain ◽  
Whole Body ◽  
Prolonged Fasting ◽  
Metabolic Gene Expression ◽  
Obese Subjects ◽  
Metabolic Inflexibility ◽  
Mitochondrial Respiratory

Obesity in humans is often associated with metabolic inflexibility, but the underlying molecular mechanisms remain incompletely understood. The aim of the present study was to investigate how adaptation to prolonged fasting affects energy/nutrient-sensing pathways and metabolic gene expression in skeletal muscle from lean and obese individuals. Twelve lean and 14 nondiabetic obese subjects were fasted for 48 h. Whole body glucose/lipid oxidation rates were determined by indirect calorimetry, and blood and skeletal muscle biopsies were collected and analyzed. In response to fasting, body weight loss was similar in both groups, but the decrease in plasma insulin and leptin and the concomitant increase in growth hormone were significantly attenuated in obese subjects. The fasting-induced shift from glucose toward lipid oxidation was also severely blunted. At the molecular level, the expression of insulin receptor-β (IRβ) was lower in skeletal muscle from obese subjects at baseline, whereas the fasting-induced reductions in insulin signaling were similar in both groups. The protein expression of mitochondrial respiratory chain components, although not modified by fasting, was significantly reduced in obese subjects. Some minor differences in metabolic gene expression were observed at baseline and in response to fasting. Surprisingly, fasting reduced AMPK activity in lean but not in obese subjects, whereas the expression of AMPK subunits was not affected. We conclude that whole body metabolic inflexibility in response to prolonged fasting in obese humans is associated with lower skeletal muscle IRβ and mitochondrial respiratory chain content as well as a blunted decline of AMPK activity.

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