Fig. 6.

Ciliogenesis of PTC124 and amlexanox treated ALMS1^{S1645*/S1645*} fibroblasts. (a-d) Representative immunofluorescent staining of cilia in (a) wild type (WT), (b) ALMS1 (untreated), (c) PTC124- and (d) amlexanox-treated ALMS1^{S1645*/S1645*} fibroblasts as detected using anti-ARL13b (shown in green) and anti-acetylated tubulin (shown in red) co-localisation. (e) Average cilia length measured in WT fibroblasts (n = 103), ALMS1 (n = 102), PTC124-treated (n = 101) and amlexanox-treated (n = 99) ALMS1^{S1645*/S1645*} fibroblasts. (f) Ciliation proportions in wild type fibroblasts (n = 103), ALMS1 (n = 102), PTC124-treated (n = 101) and amlexanox-treated (n = 99) ALMS1^{S1645*/S1645*} fibroblasts. Cilia determined by ARL13B and acetylated tubulin co-localisation were counted. P < 0.05 (*), P < 0.01 (**), P < 0.001 (***) determined by Student's t-test. Values are mean±SEM.