Fig 1: Pedigree of the family with PCD and the clinical features of the proband from this family. (A) There is one patient (the proband) in the family with PCD. Circles indicate to females. Squares indicate males. Solid symbols indicate patients. Half solid symbols indicate carriers of the identified mutations. The arrow indicates the proband. (B) Chest CT scan of the proband showed complete situs inversus, bronchiectasis and Recurrent bronchitis and pneumonia. (C) The transmission electron micrograph (TEM) of bronchial ciliary epithelium from the proband. The red arrow and the green arrow represent IDAs and ODAs, the black arrow indicates absence of IDAs and ODAs (A–C). A schematic diagram of the bronchial ciliary epithelium cross-section(D). (D–F) Immunofluorescence of bronchial ciliary epithelium revealed the existence of DNAH5, DNAH9 and DNALI1 of proband. Anti-acetylated tubulin monoclonal antibody was used to mark the ciliary axoneme. DNAH5 and DNAH9 were used to label the outer dynein arm (ODA), DNALI1 was used to label the inner dynein arm (IDA).