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. 2021 Aug 5;70:103515. doi: 10.1016/j.ebiom.2021.103515

Fig. 5.

Fig 5

Restoration of ALMS1 expression in treated ALMS1S1645*/S1645* fibroblasts. (a) Relative expression of ALMS1 in wild type (WT), ALMS1 (untreated) and PTC124- and amlexanox-treated ALMS1S1645*/S1645* fibroblasts by RT-qPCR. The levels of ALMS1 mRNA were normalised to GAPDH, ACTB and G6PD mRNA and untreated, PTC124- and amlexanox-treated levels normalised to WT levels. Experiments were performed in triplicate. (b) Quantification of ALMS1+ cells in WT (n = 112), ALMS1 (n = 114) and PTC124- (n = 101) and amlexanox-treated (n = 102) ALMS1S1645*/S1645* fibroblasts. (c-f) Representative immunofluorescent staining of cilia in (c) WT, (d) ALMS1, (e) PTC124- and (f) amlexanox-treated ALMS1S1645*/S1645* fibroblasts shown by anti-ARL13B (shown in green) staining the ciliary membrane and anti-ALMS1 (shown in red) staining in the basal body complex. P < 0.05 (*), P < 0.01 (**), P < 0.001 (***). Scale bar: 10 µm. * indicates ALMS1 expression. Values are mean±SEM, n = 3.