Mitochondrial OXPHOS function is unaffected by chronic azithromycin treatment  Shelly Ben-Harush Negari, Tzemach Aouizerat, Ariel Tenenbaum, Malena Cohen-Cymberknoh,

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Mitochondrial OXPHOS function is unaffected by chronic azithromycin treatment  Shelly Ben-Harush Negari, Tzemach Aouizerat, Ariel Tenenbaum, Malena Cohen-Cymberknoh, David Shoseyov, Eitan Kerem, Ann Saada  Journal of Cystic Fibrosis  Volume 12, Issue 6, Pages 682-687 (December 2013) DOI: 10.1016/j.jcf.2013.04.006 Copyright © 2013 European Cystic Fibrosis Society. Terms and Conditions

Fig 1 ATP production in lymphocytes isolated from stored blood. Lymphocytes from blood from a healthy adult control was isolated and frozen in freezing medium, immediately or after different time periods of storage at room temperature. Subsequently to storage in liquid nitrogen, the lymphocytes were assayed for mitochondrial ATP production capability with the different substrates; pyruvate+malate (PM), glutamate+malate (GM) or succinate (S). The results are presented as mean±SEM of at least triplicate measurements. Journal of Cystic Fibrosis 2013 12, 682-687DOI: (10.1016/j.jcf.2013.04.006) Copyright © 2013 European Cystic Fibrosis Society. Terms and Conditions

Fig. 2 ATP production in lymphocytes from patients and controls. Lymphocytes from blood from healthy adult controls (control adult), control pediatric patients (control ped) pediatric CF patients (CF) and pediatric CF patients chronically treated with azenil (CF az) and control were assayed for mitochondrial ATP production capability with different substrates; pyruvate+malate (PM), glutamate+malate (GM) or succinate (S). The results are presented as mean±SEM of at least triplicate measurements in each subject. *p<0.05. Journal of Cystic Fibrosis 2013 12, 682-687DOI: (10.1016/j.jcf.2013.04.006) Copyright © 2013 European Cystic Fibrosis Society. Terms and Conditions

Fig. 3 Enzymatic assay of ETC complexes. Lymphocytes from blood from healthy adult controls (control adult), control pediatric patients (control ped) pediatric CF patients (CF) and pediatric CF patients chronically treated with Azenil (CF az) and control were assayed for enzymatic activities of ETC complexes II–IV. Succinate dehydrogenase (SDH), succinate-cytochrome c reductase (II+III), cytochrome c oxidase (COX) and the matrix control enzyme citrate synthase. Fig. 3A depicts the actual activities while Fig. 3B depicts the relative activities compared to citrate synthase. The results are presented as mean±SEM. *p<0.05. Journal of Cystic Fibrosis 2013 12, 682-687DOI: (10.1016/j.jcf.2013.04.006) Copyright © 2013 European Cystic Fibrosis Society. Terms and Conditions