Defective motile cilia in Prickle2-deficient mice.
Motile cilia play diverse roles across phyla and cell types, and abnormalities in motile cilia lead to numerous disease states, including hydrocephalus. Although motile ciliary abnormalities in Prickle2 mutants have not yet been described, the planar cell polarity genes, including Prickle2, are implicated in the development and function of motile cilia. This report evaluates Prickle2-deficient mice for dysfunction in processes known to depend on functioning motile cilia. Prickle2-deficient mice do not develop hydrocephalus, but do display abnormal morphology and motility in the motile cilia of the ependyma. The morphology of tracheal motile cilia is also abnormal. Taken together, these results demonstrate that Prickle2 is required for normal ependymal motile cilia development and function.
Department of Neurology, The University of Iowa , Iowa City, Iowa , USA.
Levi P
LP
Sowers
Terry
T
Yin
Vinit B
VB
Mahajan
Alexander G
AG
Bassuk
D000818:Animals; D016764:Cell Polarity / Q000235:genetics; D002552:Cerebral Ventricles / Q000473:pathology / Q000648:ultrastructure; D002923:Cilia / Q000235:genetics* / Q000473:pathology* / Q000648:ultrastructure; D004805:Ependyma / Q000473:pathology / Q000648:ultrastructure; D006849:Hydrocephalus / Q000235:genetics* / Q000473:pathology*; D060588:LIM Domain Proteins / Q000172:deficiency* / Q000235:genetics; D008279:Magnetic Resonance Imaging; D008565:Membrane Proteins / Q000172:deficiency* / Q000235:genetics; D051379:Mice; D008810:Mice, Inbred C57BL; D008822:Mice, Transgenic; D046529:Microscopy, Electron, Transmission; D009154:Mutation / Q000235:genetics*
D060588:LIM Domain Proteins; D008565:Membrane Proteins; C518732:Prickle2 protein, mouse
Prickle1; Prickle2; ciliary motility; mouse ependyma; ventricle