Fig. 7.
IFT88 expression is restored in ALMS1S1645*/S1645* fibroblasts treated with TRIDs. (a-d) Representative immunofluorescent staining of cilia in (a) wild type (WT), (b) ALMS1 (untreated), (c) PTC124- and (d) amlexanox-treated ALMS1S1645*/S1645* fibroblasts shown by anti-acetylated tubulin (shown in red). IFT is detected as anti-IFT88 expression (shown in green) along the ciliary axoneme possibly accumulating at the tip. (e) The proportion of IFT88+ cells in WT fibroblasts (n = 94), ALMS1 (n = 95), PTC124-treated (n = 98) and amlexanox-treated (n = 100) ALMS1S1645*/S1645* fibroblasts. P < 0.05 (*), P < 0.01 (**), P < 0.001 (***). Scale bar: 10 µm. * indicates IFT88 expression.