Figure 2.

Effects of 5-wk oral administration of cysteamine on weight gain and lung inflammation in 2 wk-old Cftr^F508del mice. (A and B) Effects of cysteamine on weight gain. (A) Distribution of weight (g) variation of Cftr^F508del mice (n = 56) orally treated with either vehicle (n = 24) or cysteamine (n = 32) for 5 wk; °°P < 0.01 (Student t test). (B) Representative picture showing difference in body size at the end of treatment in one vehicle-treated (left) and one surviving cysteamine-treated (right) Cftr^F508del mice. Scale bar: 1 cm. (C) Effects of cysteamine on Tnf (left) and Cxcl2 (right) transcription levels in lung homogenates from 7-wk-old Cftr^F508del mice treated with either vehicle or cysteamine. Mean ± SD of triplicates of 5 surviving mice per group; °°P < 0.01 (The Student t test). (D to E) Effects of cysteamine on CFTR, and BECN1 protein levels in the lungs. (D) Mean changes of protein levels in 5 wild-type and 5 Cftr^F508del mice treated with either vehicle or cysteamine for 5 wk. Mean ± SD of 3 independent measurements; **P < 0.01 versus vehicle-treated Cftr^F508del mice (ANOVA). (E) Top, representative immunoblot with anti-CFTR (Abcam clone) and anti-BECN1 (Abcam clone Ab55878) in one mouse per treatment group. Bottom, densitometric measurement in the Cftr^F508del mouse, as percentage of vehicle-treated WT mouse normalized to TUBA/α-β tubulin levels. Mean ± SD of triplicates of independent experiments, **P < 0.01 versus vehicle-treated Cftr^F508del mice (ANOVA).