Cystic fibrosis transmembrane conductance regulator in human muscle: Dysfunction causes abnormal metabolic recovery in exercise.

@article{Lamhonwah2010CysticFT,
  title={Cystic fibrosis transmembrane conductance regulator in human muscle: Dysfunction causes abnormal metabolic recovery in exercise.},
  author={A. M. Lamhonwah and Christine E Bear and Ling Jun Huan and Patrick Kim Chiaw and Cameron A. Ackerley and Ingrid Tein},
  journal={Annals of neurology},
  year={2010},
  volume={67 6},
  pages={802-8}
}
OBJECTIVE Individuals with cystic fibrosis (CF) have exercise intolerance and skeletal muscle weakness not solely attributable to physical inactivity or pulmonary function abnormalities. CF transmembrane conductance regulator (CFTR) has been demonstrated in human bronchial smooth and cardiac muscle. Using (31)P-magnetic resonance spectroscopy of skeletal muscle, we showed CF patients to have lower resting muscle adenosine triphosphate and delayed phosphocreatine recovery times after high… CONTINUE READING
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