Figure 5.

Cysteamine reverts defective killing of P. aeruginosa through rescuing CFTR. Bone marrow-derived macrophages (BMDMs) from Cftr^{F508del/F508del} (n=3) (a–c), Cftr^−/− (n=3) (a–c), and Cftr^F508del/− (n=3) (d–f), and infected with P. aeruginosa-O1 (PAO1) for 10 min followed by 10 min (a, d) or 3 h (b–c and e–f) of culture in the presence of gentamicin. (a–b, d–e) number of CFU of PAO1; (c, f) clearance of bacteria expressed as percentage of living bacteria after 3 h of culture respect to the total amount of internalized bacteria. BMDMs from Cftr^−/− (n=3) (a–c) and Cftr^F508del/− (d–f) mice treated with 250 μM cysteamine or vehicle in the presence or absence of 3-methyl-adenine (3-MA) (3 mM) and/or CFTR_inh172 (20 μM) (d–f) and then infected with PAO1 for 10 min followed by 10 min (a, d) or 3 h (b–c and e–f) of gentamicin culture. *P<0.05, **P<0.01, Student's t-test. Means±S.D. of three independent measurements for each group of experiments. Asterisks indicate signifcant differences