scholarly journals Orphan Glutamate Receptor δ1 Subunit Required for High-Frequency Hearing

2007 ◽  
Vol 27 (12) ◽  
pp. 4500-4512 ◽  
Author(s):  
Jiangang Gao ◽  
Stéphane F. Maison ◽  
Xudong Wu ◽  
Keiko Hirose ◽  
Sherri M. Jones ◽  
...  
Keyword(s):  
Glutamate Receptor ◽  
Hair Cells ◽  
High Frequency ◽  
Outer Hair Cells ◽  
Spiral Ligament ◽  
High Frequency Hearing Loss ◽  
Type Iv ◽  
Endolymphatic Potential ◽  
And Function ◽  
Spiral Ligament Fibrocytes

ABSTRACT The function of the orphan glutamate receptor delta subunits (GluRδ1 and GluRδ2) remains unclear. GluRδ2 is expressed exclusively in the Purkinje cells of the cerebellum, and GluRδ1 is prominently expressed in inner ear hair cells and neurons of the hippocampus. We found that mice lacking the GluRδ1 protein displayed significant cochlear threshold shifts for frequencies of >16 kHz. These deficits correlated with a substantial loss of type IV spiral ligament fibrocytes and a significant reduction of endolymphatic potential in high-frequency cochlear regions. Vulnerability to acoustic injury was significantly enhanced; however, the efferent innervation of hair cells and the classic efferent inhibition of outer hair cells were unaffected. Hippocampal and vestibular morphology and function were normal. Our findings show that the orphan GluRδ1 plays an essential role in high-frequency hearing and ionic homeostasis in the basal cochlea, and the locus encoding GluRδ1 represents a candidate gene for congenital or acquired high-frequency hearing loss in humans.

scholarly journals Assessing evidence for adaptive evolution in two hearing-related genes important for high-frequency hearing in echolocating mammals

2021 ◽  
Author(s):  
Hui Wang ◽  
Hanbo Zhao ◽  
Yujia Chu ◽  
Jiang Feng ◽  
Keping Sun
Keyword(s):  
Adaptive Evolution ◽  
Hair Cells ◽  
High Frequency ◽  
Phylogenetic Trees ◽  
Outer Hair Cells ◽  
Functional Domain ◽  
Coding Region ◽  
Selective Pressures ◽  
Wide Range ◽  
Different Parts

Abstract High-frequency hearing is particularly important for echolocating bats and toothed whales. Previously, studies of the hearing-related genes Prestin, KCNQ4, and TMC1 documented that adaptive evolution of high-frequency hearing has taken place in echolocating bats and toothed whales. In this study, we present two additional candidate hearing-related genes, Shh and SK2, that may also have contributed to the evolution of echolocation in mammals. Shh is a member of the vertebrate Hedgehog gene family and is required in the specification of the mammalian cochlea. SK2 is expressed in both inner and outer hair cells, and it plays an important role in the auditory system. The coding region sequences of Shh and SK2 were obtained from a wide range of mammals with and without echolocating ability. The topologies of phylogenetic trees constructed using Shh and SK2 were different; however, multiple molecular evolutionary analyses showed that those two genes experienced different selective pressures in echolocating bats and toothed whales compared to non-echolocating mammals. In addition, several nominally significant positively selected sites were detected in the non-functional domain of the SK2 gene, indicating that different selective pressures were acting on different parts of the SK2 gene. This study has expanded our knowledge of the adaptive evolution of high-frequency hearing in echolocating mammals.

Prestin Expression in Cochlea of Bats Using Different Echolocation Systems

2014 ◽  
Vol 620 ◽  
pp. 248-252
Author(s):  
Qi Jiu Li ◽  
Xian De Zhang ◽  
Ting Ting Xu ◽  
Jiang Xia Yin
Keyword(s):  
Hair Cells ◽  
High Frequency ◽  
Low Frequency ◽  
Motor Protein ◽  
Outer Hair Cells ◽  
Intracellular Potential ◽  
First Time ◽  
Unique Ability

Outer hair cells (OHCs) have a unique ability to contract and elongate in response to changes in intracellular potential, and Prestin is the motor protein of the cochlea of the OHCs. It is the first time to invest the Prestin expression in different bat species. To invest Prestin expression in different bat species, which have different frequency, we did the coronal sections’ staining of the cochlea using immunhistochemistry. Experiment was designed to determine if the high-frequency bats’ OHCs have more expression than the low-frequency bats’OHCs. We found that the expression in three species was similar and had no obvious difference. Though the study of bats Prestin evolution suggested that Prestin has accelerating evolution in echolocation bats with high frequency, our we showed that the Prestin expression has nothing to do with the frequency, and the Prestin expression in high-frequency bats and low-frequency bats is similar.

Expression and function of channelrhodopsin 2 in mouse outer hair cells

2013 ◽  
Author(s):  
Fangyi Chen ◽  
Tao Wu ◽  
Teresa Wilson ◽  
Hrebesh Subhash ◽  
Irina Omelchenko ◽  
...  
Keyword(s):  
Hair Cells ◽  
Outer Hair Cells ◽  
And Function

scholarly journals NTPDase1 and NTPDase2 Immunolocalization in Mouse Cochlea: Implications for Regulation of P2 Receptor Signaling

2002 ◽  
Vol 50 (11) ◽  
pp. 1435-1441 ◽  
Author(s):  
Srdjan M. Vlajkovic ◽  
Peter R. Thorne ◽  
Jean Sévigny ◽  
Simon C. Robson ◽  
Gary D. Housley
Keyword(s):  
Hair Cells ◽  
Spiral Ganglion ◽  
Organ Of Corti ◽  
Nerve Fibers ◽  
Outer Hair Cells ◽  
Spiral Ligament ◽  
Receptor Signaling ◽  
P2 Receptor ◽  
Efferent Nerve ◽  
Root Region

Cellular, molecular, and physiological studies have demonstrated an important signaling role for ATP and related nucleotides acting via P2 receptors in the cochlea of the inner ear. Signal modulation is facilitated by ectonucleotidases, a heterologous family of surface-located enzymes involved in extracellular nucleotide hydrolysis. Our previous studies have implicated CD39/NTPDase1 and CD39L1/NTPDase2, members of the ectonucleoside triphosphate diphosphohydrolase (E-NTPDase) family, as major ATP-hydrolyzing enzymes in the tissues lining the cochlear endolymphatic and perilymphatic compartments. NTPDase1 hydrolyzes both nucleoside triphosphates and diphosphates. In contrast, NTPDase2 is a preferential nucleoside triphosphatase. This study characterizes expression of these E-NTPDases in the mouse cochlea by immunohistochemistry. NTPDase1 can be immunolocalized to the cochlear vasculature and neural tissues (primary auditory neurons in the spiral ganglion). In contrast, NTPDase2 immunolabeling was principally localized to synaptic regions of the sensory inner and outer hair cells, stereocilia and cuticular plates of the outer hair cells, supporting cells of the organ of Corti (Deiters’ cells and inner border cells), efferent nerve fibers located in the intraganglionic spiral bundle, and in the outer sulcus and root region of the spiral ligament. This differential expression of NTPDase1 and 2 in the cochlea suggests spatial regulation of P2 receptor signaling, potentially involving different nucleotide species and hydrolysis kinetics.

Sensory transduction and frequency selectivity in the basal turn of the guinea-pig cochlea

1992 ◽  
Vol 336 (1278) ◽  
pp. 317-324 ◽  
Keyword(s):  
Guinea Pig ◽  
Hair Cells ◽  
High Frequency ◽  
Basilar Membrane ◽  
Receptor Potential ◽  
Outer Hair Cells ◽  
Sensory Transduction ◽  
Voltage Response ◽  
Operating Range ◽  
Dc Component

Receptor potentials recorded from outer hair cells (ohc ) and inner hair cells (ihc) in the basal highfrequency turn were com pared. The dc component of the ihc receptor potential is maximized to ensure that ihcs can signal a voltage response to high-frequency tones. The ohc dc component is minimized so that ohcs transduce in the most sensitive region of their operating range. The phase and magnitude of ohc receptor potentials were recorded as an indicator of the magnitude and phase of the energy which is fed back to the basilar membrane to provide the basis for the sharp tuning and fine sensitivity of the cochlea to tones. IHC receptor potentials were recorded to assess the net effect of the feedback on the mechanics of the cochlea. It was concluded that ohcs generate feedback which enhances the ihc responses only at the best frequency. At frequencies below cf, ihc dc responses are elicited only when the ohc ac responses begin to saturate.

scholarly journals Striatin is required for hearing and affects inner hair cells and ribbon synapses

2020 ◽  
Author(s):  
Prathamesh Thangaraj Nadar Ponniah ◽  
Shahar Taiber ◽  
Michal Caspi ◽  
Tal Koffler-Brill ◽  
Amiel A. Dror ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Hair Cells ◽  
High Frequency ◽  
Cell Junctions ◽  
Scanning Electron Micrographs ◽  
High Frequency Hearing Loss ◽  
Inner Hair Cells ◽  
Brainstem Response ◽  
Deficient Mice ◽  
Cell Cell

AbstractStriatin, a subunit of the serine/threonine phosphatase PP2A, is a core member of the conserved striatin-interacting phosphatase and kinase (STRIPAK) complexes. The protein is expressed in the cell junctions between epithelial cells, which play a role in maintaining cell-cell junctional integrity. Since adhesion is crucial for the function of the mammalian inner ear, we examined the localization and function of striatin in the mouse cochlea. Our results show that in neonatal mice, striatin is specifically expressed in the cell-cell junctions of the inner hair cells, the receptor cells in the mammalian cochlea. Auditory brainstem response measurements of striatin-deficient mice indicated a progressive, high-frequency hearing loss, suggesting that striatin is essential for normal hearing. Moreover, scanning electron micrographs of the organ of Corti revealed a moderate degeneration of the outer hair cells in the middle and basal regions, concordant with the high-frequency hearing loss. Importantly, striatin-deficient mice show aberrant ribbon synapse maturation that may lead to the observed auditory impairment. Together, these results suggest a novel function for striatin in the mammalian auditory system.

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