Early Hearing Loss upon Disruption of Slc4a10 in C57BL/6 Mice
Autor: | Alena Maul, Jeppe Praetorius, Tanja Herrmann, Hannes Maier, Antje K. Huebner, Sandor Nietzsche, Christian A. Hübner |
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Rok vydání: | 2019 |
Předmět: |
bicarbonate transport
Endocochlear potential Endolymph Hearing loss Biology 01 natural sciences Connexins 03 medical and health sciences 0302 clinical medicine Hearing deafness 0103 physical sciences Fibrocyte Connexin 30 otorhinolaryngologic diseases medicine Animals Inner ear Chloride-Bicarbonate Antiporters 010301 acoustics Cochlea Mice Knockout Slc4a10 pH Sodium-Bicarbonate Symporters Bicarbonate transport Sensory Systems Mitochondria Cell biology Slc4a7 Connexin 26 Mice Inbred C57BL medicine.anatomical_structure fibrocyte Otorhinolaryngology Spiral ligament NCBE Spiral Ligament of Cochlea sense organs medicine.symptom 030217 neurology & neurosurgery Research Article |
Zdroj: | J Assoc Res Otolaryngol Huebner, A K, Maier, H, Maul, A, Nietzsche, S, Herrmann, T, Praetorius, J & Hübner, C A 2019, ' Early Hearing Loss upon Disruption of Slc4a10 in C57BL/6 Mice ', Journal of the Association for Research in Otolaryngology, vol. 20, no. 3, pp. 233-245 . https://doi.org/10.1007/s10162-019-00719-1 |
ISSN: | 1438-7573 1525-3961 |
Popis: | The unique composition of the endolymph with a high extracellular K + concentration is essential for sensory transduction in the inner ear. It is secreted by a specialized epithelium, the stria vascularis, that is connected to the fibrocyte meshwork of the spiral ligament in the lateral wall of the cochlea via gap junctions. In this study, we show that in mice the expression of the bicarbonate transporter Slc4a10/Ncbe/Nbcn2 in spiral ligament fibrocytes starts shortly before hearing onset. Its disruption in a C57BL/6 background results in early onset progressive hearing loss. This hearing loss is characterized by a reduced endocochlear potential from hearing onset onward and progressive degeneration of outer hair cells. Notably, the expression of a related bicarbonate transporter, i.e., Slc4a7/Nbcn1, is also lost in spiral ligament fibrocytes of Slc4a10 knockout mice. The histological analysis of the spiral ligament of Slc4a10 knockout mice does not reveal overt fibrocyte loss as reported for Slc4a7 knockout mice. The ultrastructural analysis, however, shows mitochondrial alterations in fibrocytes of Slc4a10 knockout mice. Our data suggest that Slc4a10 and Slc4a7 are functionally related and essential for inner ear homeostasis. |
Databáze: | OpenAIRE |
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