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The Journal of Neuroscience, August 11, 2004, 24(32):7051-7062; doi:10.1523/JNEUROSCI.1640-04.2004

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Cellular/Molecular
Deafness in Claudin 11-Null Mice Reveals the Critical Contribution of Basal Cell Tight Junctions to Stria Vascularis Function

Alexander Gow,¹ Caroline Davies,² Cherie M. Southwood,¹ Gregory Frolenkov,² Mark Chrustowski,¹ Lily Ng,³ Daisuke Yamauchi,⁴ Daniel C. Marcus,⁴ and Bechara Kachar²

¹Center for Molecular Medicine and Genetics, Carman and Ann Adams Department of Pediatrics, Department of Neurology, Wayne State University, Detroit, Michigan 48201, ²Section on Structural Biology, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, Maryland 20892, ³Department of Genetics, Mount Sinai School of Medicine, New York, New York 10029, and ⁴Department of Anatomy and Physiology, Kansas State University, Manhattan, Kansas 66506

Generation of a strong electrical potential in the cochlea is uniquely mammalian and may reflect recent evolutionary advances in cellular voltage-dependent amplifiers. This endocochlear potential is hypothesized to dramatically improve hearing sensitivity, a concept that is difficult to explore experimentally, because manipulating cochlear function frequently causes rapid degenerative changes early in development. Here, we examine the deafness phenotype in adult Claudin 11-null mice, which lack the basal cell tight junctions that give rise to the intrastrial compartment and find little evidence of cochlear pathology. Potassium ion recycling is normal in these mutants, but endocochlear potentials were below 30 mV and hearing thresholds were elevated 50 dB sound pressure level across the frequency spectrum. Together, these data demonstrate the central importance of basal cell tight junctions in the stria vascularis and directly verify the two-cell hypothesis for generation of endocochlear potential. Furthermore, these data indicate that endocochlear potential is an essential component of the power source for the mammalian cochlear amplifier.

Key words: evoked potentials; targeted deletion; oligodendrocyte-specific protein; -galactosidase; freeze fracture; homologous recombination

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Received April 29, 2004; revised June 9, 2004; accepted June 9, 2004.

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