An important practical implication of our work is that DNAAF6 Q171∗, a mutant of DNAAF6 found in a PCD patient, could not interact with LARP6 and not restore defects of Xenopus DNAAF6 morphants, such as morphology and motility of cilia in Xenopus epidermal MCCs. Here, DNAAF6 is linked to primary ciliary dyskinesia.