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Updated: Aug 14, 2026
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Evaluation of Planar-Cell-Polarity Phenotypes in Ciliopathy Mouse Mutant Cochlea
Published on: February 21, 2016
Molecular mechanisms underlying inner ear patterning defects in kreisler mutants
Daniel Choo1, Jaye Ward, Alisa Reece
1Department of Otolaryngology Head and Neck Surgery, Center for Hearing and Deafness Research, University of Cincinnati College of Medicine, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, OH 45229-3039, USA. daniel.choo@cchmc.org
Developmental Biology
|December 6, 2005
Summary
The kr/mafB gene mutation in mice causes inner ear defects by disrupting hindbrain development. This study reveals key gene changes affecting inner ear structure and sensory organ development.
Area of Science:
- Developmental Biology
- Genetics
- Neuroscience
Background:
- Mutations in the kr/mafB gene are linked to early inner ear defects.
- These defects correlate with abnormal hindbrain development and signaling.
Purpose of the Study:
- To investigate the role of kr/mafB and hindbrain development in inner ear patterning.
- To analyze ear morphogenesis and gene expression in kreisler mutants.
Main Methods:
- Utilized a paint-fill technique to examine ear morphogenesis in kreisler mice.
- Assessed expression patterns of key developmental genes within the otocyst.
Main Results:
- Loss of dorsal otic structures (endolymphatic duct/sac) linked to downregulated Gbx2, Dlx5, and Wnt2b.
- Expanded ventral Otx2 expression correlates with cochlear defects.
- Sensory organ development is significantly disrupted.
Conclusions:
- Hindbrain cues, indirectly influenced by kr/mafB, are crucial for inner ear morphogenesis.
- Gbx2, Dlx5, Wnt2b, and Otx2 are identified as critical otic genes affected by the kr/mafB-hindbrain pathway disruption.
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