Back to Search
Start Over
Early Hearing Loss upon Disruption of Slc4a10 in C57BL/6 Mice
- Source :
- 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
- Publication Year :
- 2019
- Publisher :
- Springer Science and Business Media LLC, 2019.
-
Abstract
- 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.
- Subjects :
- 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
Subjects
Details
- ISSN :
- 14387573 and 15253961
- Volume :
- 20
- Database :
- OpenAIRE
- Journal :
- Journal of the Association for Research in Otolaryngology
- Accession number :
- edsair.doi.dedup.....47c9e2d2da0a0a45705be7e40557bf86