Phenotype Image Detail

Image

Caption

Inner ear morphology, hearing thresholds, and otoconia of Zeb1^Tw/Zeb1⁺(Tw/+) and Zeb1^Tw/Zeb1^Tw(Tw/Tw) mice. Paint-filled inner ears of wild-type, heterozygous and homozygous mice at E14.5 are shown. Lateral views (A-C) show the entire cochlea, vestibular labyrinth and endolymphatic sac. Medial views (D-F) show the non-ampullated end of the lateral semicircular canal. Ventral views (G-I) show the cochlear duct. The overall structure of Zeb1^Tw/Zeb1⁺ inner ears was intact, but the contours of the semicircular canals were irregular due to small bulges and projections along the canals (indicated by asterisks in B). There were irregularities and constrictions of the non-ampullated ends of lateral canals (E). Shortened cochlear ducts are consistently observed in Zeb1^Tw/Zeb1⁺ ears (H). Zeb1^Tw/Zeb1^Tw inner ear anatomy is disrupted but recognizable (C, F, I). Zeb1^Tw/Zeb1^Tw semicircular canals and cochlear ducts were either discontinuous or ruptured. Average ABR thresholds for all wild-type ears (n = 24) are shown as white circles and heterozygous mutant ears (n = 24) as black circles with the standard error of the mean (SEM). Results are shown for click, 8-, 16- and 32-kHz pure-tone stimuli (J). Scanning electron microscopy showed no difference in otoconia between wild-type (K) and heterozygous mutant saccules (L) at P6. Heterozygous mutant utricles (N) had giant otoconia, a, anterior semicircular canal; c, cochlear duct; es, endolymphatic sac; l, lateral semicircular canal; p, posterior semicircular canal; s, saccule; u, utricle.

Copyright

This image is from Kurima K, PLoS Genet 2011 Sep;7(9):e1002307, and is displayed under the terms of the Creative Commons Attribution 4.0 International License. J:177274

Associated Alleles

Symbol	Name
Zeb1^Tw	zinc finger E-box binding homeobox 1; twirler

Associated Genotypes

Allelic Composition	Genetic Background
Zeb1^Tw/Zeb1^Tw	B6.Cg-Zeb1^Tw
Zeb1^Tw/Zeb1^+	B6.Cg-Zeb1^Tw