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Alterations of skeletal muscle bioenergetics in a mouse with F508del mutation leading to a cystic fibrosis-like condition

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 mitochondrial dysfunction. Studies were performed on congenic C57BL/6J and F508del (Cftr...

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Publicat a:Am J Physiol Endocrinol Metab
Autors principals: Lai, Nicola, Kummitha, Chinna, Drumm, Mitchell, Hoppel, Charles
Format: Artigo
Idioma:Inglês
Publicat: American Physiological Society 2019
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Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC6732463/
https://ncbi.nlm.nih.gov/pubmed/31211618
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1152/ajpendo.00064.2019
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