Lily Paemka, Brian N. McCullagh, Mahmoud H. Abou Alaiwa, David A

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Monocyte derived macrophages from CF pigs exhibit increased inflammatory responses at birth  Lily Paemka, Brian N. McCullagh, Mahmoud H. Abou Alaiwa, David A. Stoltz, Qian Dong, Christoph O. Randak, Robert D. Gray, Paul B. McCray  Journal of Cystic Fibrosis  Volume 16, Issue 4, Pages 471-474 (July 2017) DOI: 10.1016/j.jcf.2017.03.007 Copyright © 2017 The Authors Terms and Conditions

Fig. 1 CFTR is expressed in monocyte derived macrophages. A, B. MDMs from non-CF and CF pigs have characteristic macrophage morphology and were phenotypically identical (scale bar=20μM). C. Quantitative rtPCR was carried out to assess CFTR mRNA abundance in non-CF porcine primary airway epithelia (n=10) and MDMs (n=7) (*P<0.001, Mann–Whitney rank sum test). CFTR is functional in non-CF MDMs. D. Whole-cell current recorded in response to 10μM forskolin (Fsk) and 100μM 3-isobutyl-2-methylxanthine (IBMX, upper panel) and after adding 100μM of CFTR inhibitor GlyH-101 (GlyH, lower panel) to the bath solution. Example of currents from one non-CF macrophage shown. Voltage-pulse protocol used for whole cell patch clamping is shown in inset on left. Current–voltage relationship of the GlyH-sensitive current is shown in E. F. GlyH-sensitive Fsk/IBMX-activated whole-cell current is absent in CF MDMs (holding potential=−70mV, n=5 non-CF and 3 CF MDMs, *P<0.05 Mann–Whitney rank sum test). Journal of Cystic Fibrosis 2017 16, 471-474DOI: (10.1016/j.jcf.2017.03.007) Copyright © 2017 The Authors Terms and Conditions

Fig. 2 CF MDMs exhibit increased cytokine release in response to LPS stimulation. MDMs from newborn CF pigs release more IL8 (A) and TNFα (B) compared to non-CF in a dose-dependent manner (n=8 non-CF and 8 CF, *P<0.05, **P<0.01, 1 way ANOVA with Newman–Keuls multiple comparison test). Journal of Cystic Fibrosis 2017 16, 471-474DOI: (10.1016/j.jcf.2017.03.007) Copyright © 2017 The Authors Terms and Conditions