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Caption Vestibular histopathology of the Kcnq1tm1Apf/Kcnq1tm1Apf mouse. A: Normal histology of the urticle of unaffected Kcnq1tm1Apf/Kcnq1+ mouse for comparison. B: Affected Kcnq1tm1Apf/Kcnq1tm1Apf 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
Symbol Name
Kcnq1tm1Apf potassium voltage-gated channel, subfamily Q, member 1; targeted mutation 1, Andrew P Feinberg
Allelic Composition Genetic Background
Kcnq1tm1Apf/Kcnq1tm1Apf involves: 129P2/OlaHsd * C57BL/6

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