Phenotype Image Detail

Image

Caption

Vestibular histopathology of the Kcnq1^tm1Apf/Kcnq1^tm1Apf mouse. A: Normal histology of the urticle of unaffected Kcnq1^tm1Apf/Kcnq1⁺ mouse for comparison. B: Affected Kcnq1^tm1Apf/Kcnq1^tm1Apf mouse, showing marked reduction in the size of the endolymphatic compartment, associated with adhesions between the membranous envelope, the inner surface of the otic capsule and the otolithic membrane. The otolithic membrane shows irregular borders and is separated from the neuroepithelium (filled arrows). There is also a loculated collection of granular material (open arrows) in close association with the otolithic membrane. There is a marked reduction in hair cell number (identified by the presence of a white halo around the nucleus) in the neuroepithelium of the utricle in the mutant mouse (asterisk). These cells are particularly prominent in the center of the neuroepithelium in the heterozygous mouse in which type I cells with large chalice-like nerve endings predominate.

Copyright

This image is from Lee MP, J Clin Invest 2000 Dec;106(12):1447-55 and is displayed with the permission of the American Society for Clinical Investigation who owns the Copyright. J:66428

Associated Alleles

Symbol	Name
Kcnq1^tm1Apf	potassium voltage-gated channel, subfamily Q, member 1; targeted mutation 1, Andrew P Feinberg

Associated Genotypes

Allelic Composition	Genetic Background
Kcnq1^tm1Apf/Kcnq1^tm1Apf	involves: 129P2/OlaHsd * C57BL/6