The homozygous conditional Odf2-KO (Odf2ΔΕx6,7/ΔΕx6,7) mice lacking exons 6 and 7 of the Odf2 gene, which encodes centrosome-associated protein Odf2/cenexin, have defects in the structure and function of basal bodies in the ciliated tissues, causing primary ciliary dyskinesia, such as coughing in the trachea and early postnatal death by impaired gastrointestinal motility (possibly Hirschsprung’s disease), but the effect on the sperm remains unclear21. Here, ODF2 is linked to Hirschsprung disease.