Transmission electron microscope analysis of the bronchial epithelium samples from PCD patients with <i>RSPH4A</i> mutations.

<p><b>A</b> Ciliary cross sections – magnification 30,000 (patient #181). <i>Left panel</i> – a typical picture of proximal ciliary cross sections (cilia are accompanied by numerous microvilli); <i>right panel</i> – ciliary cross sections taken at the distance from the cell membrane (no microvilli present); <b>B</b> Examples of MT defects in patients #181, #337 and #340 (a blown-up view). <b>C</b> Longitudinal sections of axonemes (patient #337); <i>left panel</i>: magnification 16,000; <i>right panel</i> – a blown up view of a single cilium. White, black and hashed arrows indicate 9+2, 9+0 and 8+1 MT arrangement, respectively.