Rebecca J. Darrah, Ilya R. Bederman, Anna L. Mitchell, Craig A

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Ventilatory pattern and energy expenditure are altered in cystic fibrosis mice Rebecca J. Darrah, Ilya R. Bederman, Anna L. Mitchell, Craig A. Hodges, Cara K. Campanaro, Mitchell L. Drumm, Frank J. Jacono Journal of Cystic Fibrosis Volume 12, Issue 4, Pages 345-351 (July 2013) DOI: 10.1016/j.jcf.2012.11.008 Copyright © 2012 European Cystic Fibrosis Society. Terms and Conditions

Fig. 1 Ventilatory pattern and frequency are altered in CF mice. (A) Representative tracings of breathing pattern of non-CF (C57BL/6J) mice (top), congenic CF F508del mice (middle) and congenic CF F508del gut-corrected mice (bottom). CF mice (F508del and F508del gut-corrected) had a higher respiratory rate (B) and coefficient of variation of respiratory cycle length (C) than non-CF (C57BL/6J) mice. *p<0.05 compared to non-CF (C57BL/6J) mice. Journal of Cystic Fibrosis 2013 12, 345-351DOI: (10.1016/j.jcf.2012.11.008) Copyright © 2012 European Cystic Fibrosis Society. Terms and Conditions

Fig. 2 (A) Autocorrelation coefficient was decreased at the average cycle length for CF mice as compared to non-CF mice. These data are consistent with the increase in the coefficient of variation. (B) An index of nonlinear complexity was similar between all three groups of mice. *p<0.05 compared to non-CF (C57BL/6J) mice. Journal of Cystic Fibrosis 2013 12, 345-351DOI: (10.1016/j.jcf.2012.11.008) Copyright © 2012 European Cystic Fibrosis Society. Terms and Conditions

Fig. 3 24-hour profiles for carbon dioxide production (A), oxygen consumption (B) and total energy expenditure (C) per kg body weight are shown for non-CF (C57BL/6J) (black squares), CF F508del (white squares) and CF F508del gut-corrected (gray squares) mice. Data represent average values±standard deviation for all animals at each time point. CF F508del mice had significantly elevated energy expenditure in both the dark and light phases, and across the 24-hour time period, as quantified in Fig. 4. Journal of Cystic Fibrosis 2013 12, 345-351DOI: (10.1016/j.jcf.2012.11.008) Copyright © 2012 European Cystic Fibrosis Society. Terms and Conditions

Fig. 4 Average total energy expenditure per kg body weight is shown for the total 24-hour period, and for the dark (active) and light (rest) phases for non-CF (C57BL/6J) (black bars), CF F508del (white bars) and CF F508del gut-corrected (gray bars) mice. Data represent average values±standard deviation. *p<0.05 compared to non-CF (C57BL/6J) mice; #p<0.05 compared to CF F508del gut-corrected mice. Journal of Cystic Fibrosis 2013 12, 345-351DOI: (10.1016/j.jcf.2012.11.008) Copyright © 2012 European Cystic Fibrosis Society. Terms and Conditions