FIGURE 7.

In vitro ciliary transport is impaired in CFAP46- and CFAP54-variant individuals. a) To assess the ciliary transport in vitro, respiratory epithelial cells from healthy control individuals and from CFAP46- and CFAP54-variant individuals were cultured at the air–liquid interface (ALI). After 30 days of differentiation, the ALI-cultured respiratory cell layers were used for ciliary transport assays, evaluating the transport of fluorescent particles by ciliary beating from the top view. b) The mean ciliary beat frequencies (CBFs) (indicated by the black bars) in OP-64 II2, OP-1245 II1 and OP-1822 II1 did not differ significantly from healthy controls (p=0.62, t-test). c) In contrast, the mean velocities of fluorescent particles were significantly reduced in the ALI-inserts from OP-64 II2 (37.6%), OP-1245 II1 (53.1%) and OP-1822 II1 (30.6%) (p=0.00022, t-test). Because two different microscope settings were used for the analysis, the data were normalised based on the healthy control pool (Nikon: triangles, Leica: circles). The exact values for CBF (Hz) and ciliary transport velocity (µm·s⁻¹) are shown in supplementary figure S6. Significant differences are indicated. ***: p<0.001.