The defects of hair-bundle orientation in both bpck and Tmem67−/− lines are similar to those observed in mouse models of the human ciliopathies, such as the Alström syndrome (Jagger et al., 2011), BBS (May-Simera et al., 2009; Ross et al., 2005), and the Kif3a ciliary mutant (Sipe and Lu, 2011). This evidence concerns the gene TMEM67 and ciliopathy.