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Research Article

Lack of CFTR in Skeletal Muscle Predisposes to Muscle Wasting and Diaphragm Muscle Pump Failure in Cystic Fibrosis Mice

  • Maziar Divangahi equal contributor,

    equal contributor Contributed equally to this work with: Maziar Divangahi, Haouaria Balghi

    Affiliation: Meakins-Christie Laboratories and Respiratory Division, McGill University Health Centre and Research Institute, Montreal, Quebec, Canada

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  • Haouaria Balghi equal contributor,

    equal contributor Contributed equally to this work with: Maziar Divangahi, Haouaria Balghi

    Affiliation: Meakins-Christie Laboratories and Respiratory Division, McGill University Health Centre and Research Institute, Montreal, Quebec, Canada

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  • Gawiyou Danialou,

    Affiliation: Meakins-Christie Laboratories and Respiratory Division, McGill University Health Centre and Research Institute, Montreal, Quebec, Canada

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  • Alain S. Comtois,

    Affiliation: Meakins-Christie Laboratories and Respiratory Division, McGill University Health Centre and Research Institute, Montreal, Quebec, Canada

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  • Alexandre Demoule,

    Affiliations: Meakins-Christie Laboratories and Respiratory Division, McGill University Health Centre and Research Institute, Montreal, Quebec, Canada, Université Paris 6 Pierre et Marie Curie, UPRES EA2397, Paris, France

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  • Sheila Ernest,

    Affiliation: Meakins-Christie Laboratories and Respiratory Division, McGill University Health Centre and Research Institute, Montreal, Quebec, Canada

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  • Christina Haston,

    Affiliation: Meakins-Christie Laboratories and Respiratory Division, McGill University Health Centre and Research Institute, Montreal, Quebec, Canada

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  • Renaud Robert,

    Affiliation: Department of Physiology, McGill University, Montreal, Quebec, Canada

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  • John W. Hanrahan,

    Affiliation: Department of Physiology, McGill University, Montreal, Quebec, Canada

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  • Danuta Radzioch,

    Affiliation: Centre for the Study of Host Resistance, Departments of Experimental Medicine and Human Genetics, McGill University Health Centre Research Institute, Montreal, Quebec, Canada

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  • Basil J. Petrof mail

    basil.petrof@mcgill.ca

    Affiliation: Meakins-Christie Laboratories and Respiratory Division, McGill University Health Centre and Research Institute, Montreal, Quebec, Canada

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  • Published: July 31, 2009
  • DOI: 10.1371/journal.pgen.1000586

About the Authors

Maziar Divangahi, Haouaria Balghi, Gawiyou Danialou, Alain S. Comtois, Alexandre Demoule, Sheila Ernest, Christina Haston, Basil J. Petrof
Meakins-Christie Laboratories and Respiratory Division, McGill University Health Centre and Research Institute, Montreal, Quebec, Canada
Alexandre Demoule
Université Paris 6 Pierre et Marie Curie, UPRES EA2397, Paris, France
Renaud Robert, John W. Hanrahan
Department of Physiology, McGill University, Montreal, Quebec, Canada
Danuta Radzioch
Centre for the Study of Host Resistance, Departments of Experimental Medicine and Human Genetics, McGill University Health Centre Research Institute, Montreal, Quebec, Canada

Corresponding Author

Email: basil.petrof@mcgill.ca

Competing Interests

The authors have declared that no competing interests exist.

Author Contributions

Conceived and designed the experiments: MD HB BJP. Performed the experiments: MD HB GD ASC AD SE RR. Analyzed the data: MD HB GD ASC AD SE BJP. Contributed reagents/materials/analysis tools: CH RR JWH DR BJP. Wrote the paper: MD HB BJP.