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CrossTalk proposal: Skeletal muscle oxidative capacity is altered in patients with cystic fibrosis
Author(s) -
RodriguezMiguelez Paula,
Erickson Melissa L.,
McCully Kevin K.,
Harris Ryan A.
Publication year - 2017
Publication title -
the journal of physiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.802
H-Index - 240
eISSN - 1469-7793
pISSN - 0022-3751
DOI - 10.1113/jp272486
Subject(s) - cystic fibrosis , skeletal muscle , exercise intolerance , medicine , endocrinology , population , heart failure , environmental health
Cystic fibrosis (CF) is an autosomal recessive disorder characterized by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, which functions as an ATP-gated anion channel. Pulmonary dysfunction is the most common clinical manifestation of CF; however, there are many other systemic consequences that contribute to the shortened life expectancy in this population. Exercise intolerance has been shown to predict mortality in patients with CF independent of lung function. Despite the clinical importance for assessing exercise capacity, the mechanisms which lead to exercise intolerance in CF have yet to be elucidated. Although evidence suggests that the impaired expression of CFTR in skeletal muscle may be associated with abnormalities in muscle oxygenation and muscle metabolism, there are recent studies that have described a preserved muscle metabolism in patients with CF. Considering the available data, support of