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Deafness in Claudin 11-null mice reveals the critical contribution of basal cell tight junctions to stria vascularis function.
J Neurosci. 2004 Aug 11; 24(32):7051-62.JN

Abstract

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.

Authors+Show Affiliations

Center for Molecular Medicine and Genetics, Carman and Ann Adams Department of Pediatrics, Wayne State University, Detroit, Michigan 48201, USA. agow@genetics.wayne.eduNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, U.S. Gov't, P.H.S.

Language

eng

PubMed ID

15306639

Citation

Gow, Alexander, et al. "Deafness in Claudin 11-null Mice Reveals the Critical Contribution of Basal Cell Tight Junctions to Stria Vascularis Function." The Journal of Neuroscience : the Official Journal of the Society for Neuroscience, vol. 24, no. 32, 2004, pp. 7051-62.
Gow A, Davies C, Southwood CM, et al. Deafness in Claudin 11-null mice reveals the critical contribution of basal cell tight junctions to stria vascularis function. J Neurosci. 2004;24(32):7051-62.
Gow, A., Davies, C., Southwood, C. M., Frolenkov, G., Chrustowski, M., Ng, L., Yamauchi, D., Marcus, D. C., & Kachar, B. (2004). Deafness in Claudin 11-null mice reveals the critical contribution of basal cell tight junctions to stria vascularis function. The Journal of Neuroscience : the Official Journal of the Society for Neuroscience, 24(32), 7051-62.
Gow A, et al. Deafness in Claudin 11-null Mice Reveals the Critical Contribution of Basal Cell Tight Junctions to Stria Vascularis Function. J Neurosci. 2004 Aug 11;24(32):7051-62. PubMed PMID: 15306639.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Deafness in Claudin 11-null mice reveals the critical contribution of basal cell tight junctions to stria vascularis function. AU - Gow,Alexander, AU - Davies,Caroline, AU - Southwood,Cherie M, AU - Frolenkov,Gregory, AU - Chrustowski,Mark, AU - Ng,Lily, AU - Yamauchi,Daisuke, AU - Marcus,Daniel C, AU - Kachar,Bechara, PY - 2004/8/13/pubmed PY - 2005/1/26/medline PY - 2004/8/13/entrez SP - 7051 EP - 62 JF - The Journal of neuroscience : the official journal of the Society for Neuroscience JO - J Neurosci VL - 24 IS - 32 N2 - 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. SN - 1529-2401 UR - https://www.unboundmedicine.com/medline/citation/15306639/Deafness_in_Claudin_11_null_mice_reveals_the_critical_contribution_of_basal_cell_tight_junctions_to_stria_vascularis_function_ L2 - http://www.jneurosci.org/cgi/pmidlookup?view=long&pmid=15306639 DB - PRIME DP - Unbound Medicine ER -