Abstract:
:The intercellular gap junction channels formed by connexins (CXs) are important for recycling potassium ions in the inner ear. CXs are encoded by a family of the CX gene, such as GJB2, and the mechanism leading to mutant connexin-associated diseases, including hearing loss, remains to be elucidated. In this study, using bioinformatics, we found that two zebrafish cx genes, cx27.5 and cx30.3, are likely homologous to human and mouse GJB2. During embryogenesis, zebrafish cx27.5 was rarely expressed at 1.5-3 h post-fertilization (hpf), but a relatively high level of cx27.5 expression was detected from 6 to 96 hpf. However, zebrafish cx30.3 transcripts were hardly detected until 9 hpf. The temporal experiment was conducted in whole larvae. Both cx27.5 and cx30.3 transcripts were revealed significantly in the inner ear by reverse transcription polymerase chain reaction (RT-PCR) and whole-mount in situ hybridization (WISH). In the HeLa cell model, we found that zebrafish Cx27.5 was distributed intracellularly in the cytoplasm, whereas Cx30.3 was localized in the plasma membrane of HeLa cells stably expressing Cx proteins. The expression pattern of zebrafish Cx30.3 in HeLa cells was more similar to that of cells expressing human CX26 than Cx27.5. In addition, we found that Cx30.3 was localized in the cell membrane of hair cells within the inner ear by immunohistochemistry (IHC), suggesting that zebrafish cx30.3 might play an essential role in the development of the inner ear, in the same manner as human GJB2. We then performed morpholino knockdown studies in zebrafish embryos to elucidate the physiological functions of Cx30.3. The zebrafish cx30.3 morphants exhibited wild-type-like and heart edema phenotypes with smaller inner ears at 72 hpf. Based on these results, we suggest that the zebrafish Cx30.3 and mammalian CX26 may play alike roles in the inner ear. Thus, zebrafish can potentially serve as a model for studying hearing loss disorders that result from human CX26 mutations.
journal_name
Hear Resjournal_title
Hearing researchauthors
Chang-Chien J,Yen YC,Chien KH,Li SY,Hsu TC,Yang JJdoi
10.1016/j.heares.2014.04.010subject
Has Abstractpub_date
2014-07-01 00:00:00pages
55-66eissn
0378-5955issn
1878-5891pii
S0378-5955(14)00076-8journal_volume
313pub_type
杂志文章相关文献
HEARING RESEARCH文献大全abstract::The vibration characteristics (amplitude and phase as a function of frequency) of the tympanic membrane in the grass frog were measured using a laser-doppler velocity meter. It was tested to what extent the frog's acoustic system behaves as a pressure gradient receiver. This might clarify how the frog localizes sound....
journal_title:Hearing research
pub_type: 杂志文章
doi:10.1016/0378-5955(84)90018-2
更新日期:1984-05-01 00:00:00
abstract::Tinnitus is highly prevalent in the general population. Tinnitus sufferers often report having difficulties focusing on a task at hand and ignoring the tinnitus percept. Behavioral studies have shown evidence for impairments in attention, interference inhibition, and various other executive functions in tinnitus. Howe...
journal_title:Hearing research
pub_type: 杂志文章
doi:10.1016/j.heares.2015.03.006
更新日期:2015-08-01 00:00:00
abstract::The present study examined synaptic potentials of neurons in inferior colliculus (IC) cortex slice and the roles of GABA and glutamate receptors in generating these potentials. Multipolar (82%) and elongated (18%) cells were observed with intracellular biocytin staining. Electrical stimulation of the IC commissure (Co...
journal_title:Hearing research
pub_type: 杂志文章
doi:10.1016/s0378-5955(99)00129-x
更新日期:1999-11-01 00:00:00
abstract::The effect of glutathione co-therapy on the expression of gentamicin ototoxicity was tested in pigmented guinea pigs. The first group of animals was injected with gentamicin (100 mg/kg body weight/day) for two weeks followed by 10 weeks of rest. A second group received glutathione by gastric gavage immediately prior t...
journal_title:Hearing research
pub_type: 杂志文章
doi:10.1016/0378-5955(94)90255-0
更新日期:1994-06-15 00:00:00
abstract::Auditory brainstem implants (ABIs) can restore useful hearing to persons with deafness who cannot benefit from cochlear implants. However, the quality of hearing restored by ABIs rarely is comparable to that provided by cochlear implants in persons for whom those are appropriate. In an animal model, we evaluated eleme...
journal_title:Hearing research
pub_type: 杂志文章
doi:10.1016/j.heares.2018.03.009
更新日期:2018-06-01 00:00:00
abstract::Recently, many lines of evidence have supported the possibilities that vasopressin (VP) is closely linked to the formation of endolymphatic hydrops in Meniere's disease. In the present study, it was examined whether or not the chronic administration of VP might induce endolymphatic hydrops. For this purpose, histologi...
journal_title:Hearing research
pub_type: 杂志文章
doi:10.1016/s0378-5955(99)00180-x
更新日期:2000-02-01 00:00:00
abstract::In response to low-intensity, low-frequency, phase-locked tonal stimuli with non-alternating polarity, the time-average round window (RW) response of the gerbil is a mixture of the auditory nerve neurophonic (ANN) and cochlear microphonic (CM), with the former often being of equal or greater magnitude than the latter....
journal_title:Hearing research
pub_type: 杂志文章
doi:10.1016/s0378-5955(96)00195-5
更新日期:1997-02-01 00:00:00
abstract::Preservation of residual hearing after cochlear implantation is now considered an important goal of surgery. However, studies indicate an average post-operative hearing loss of around 20 dB at low frequencies. One factor which may contribute to post-operative hearing loss, but which has received little attention in th...
journal_title:Hearing research
pub_type: 杂志文章
doi:10.1016/j.heares.2016.08.006
更新日期:2016-11-01 00:00:00
abstract::Spatial hearing skills are essential for children as they grow, learn and play. These skills provide critical cues for determining the locations of sources in the environment, and enable segregation of important sounds, such as speech, from background maskers or interferers. Spatial hearing depends on availability of ...
journal_title:Hearing research
pub_type: 杂志文章,评审
doi:10.1016/j.heares.2016.01.003
更新日期:2016-08-01 00:00:00
abstract::In order to clarify the otoconia formation and turnover, tetracycline, an antibiotic that precipitates at calcifying fronts and serves as a fluorescent marker, was injected into eggs at different stages of chick embryonic development, as well as into postnatal chicken and into adult animals. The changes in the intensi...
journal_title:Hearing research
pub_type: 杂志文章
doi:10.1016/s0378-5955(00)00094-0
更新日期:2000-10-01 00:00:00
abstract::Input-specific remodeling is observed both in the primary auditory cortex (AI) and the ventral division of the medial geniculate body of the thalamus (MGBv) through motivation such as learning. Here, we show the role of AI in the MGBv remodeling induced by the electrical stimulation (ES) of the central division of the...
journal_title:Hearing research
pub_type: 杂志文章
doi:10.1016/j.heares.2015.06.016
更新日期:2015-10-01 00:00:00
abstract::The acoustic distortion product 2f1-f2 was measured in the ear canal of the gray short-tailed opossum. Monodelphis domestica, during stimulation with two pure tone stimuli of the frequencies f1 and f2 (f1 < f2). From 2f1-2f growth functions for different primary frequencies from 4 to 85 kHz, a distortion threshold cur...
journal_title:Hearing research
pub_type: 杂志文章
doi:10.1016/0378-5955(95)00232-4
更新日期:1996-05-01 00:00:00
abstract::Cochlear Implant (CI) sound coding strategies based on simultaneous stimulation lead to an increased loudness percept when compared to sequential stimulation using the same current levels. This is due to loudness summation as a result of channel interactions. Studying the loudness perception evoked by dual-channels co...
journal_title:Hearing research
pub_type: 杂志文章
doi:10.1016/j.heares.2020.108091
更新日期:2020-12-01 00:00:00
abstract::A field potential could be recorded in the deep layers of the superior colliculus (SCd) in the guinea pig following presentation of pure tone bursts at frequencies between 1 and 22 kHz, bursts of white noise, and clicks presented in the free field. The potentials evoked by these stimuli at different intensities had tw...
journal_title:Hearing research
pub_type: 杂志文章
doi:10.1016/0378-5955(87)90118-3
更新日期:1987-12-01 00:00:00
abstract::High dose of salicylate, the active ingredient in aspirin, has long been known to induce transient hearing loss, tinnitus and hyperacusis making it a powerful experimental tool. These salicylate-induced perceptual disturbances are associated with a massive reduction in the neural output of the cochlea. Paradoxically, ...
journal_title:Hearing research
pub_type: 杂志文章
doi:10.1016/j.heares.2016.10.021
更新日期:2017-04-01 00:00:00
abstract::Using single-unit electrophysiological methods we have mapped sound frequency (or cochleotopic) representation in the auditory cortex of the chinchilla. We describe the surgical approach to expose this area. We report on maps from six subjects and note a considerable variation in shape between individuals. In general,...
journal_title:Hearing research
pub_type: 杂志文章
doi:10.1016/0378-5955(96)00120-7
更新日期:1996-10-01 00:00:00
abstract::Laser Doppler vibrometry was used to measure the sound-induced tympanic membrane (TM) velocity, assessed near the umbo, in 56 normal hearing human subjects at nine sound frequencies. A second series of measurements was made in 47 subjects with sensorineural hearing loss (SNHL). Each set of measurements has features in...
journal_title:Hearing research
pub_type: 杂志文章
doi:10.1016/s0378-5955(03)00332-0
更新日期:2004-01-01 00:00:00
abstract::This paper will review some of the functional changes that occur in the central auditory pathway after the cochlea is damaged by acoustic overstimulation or by carboplatin, an ototoxic drug that selectively destroys inner hair cells (IHCs) in the chinchilla. Acoustic trauma typically impairs the sensitivity and tuning...
journal_title:Hearing research
pub_type: 杂志文章,评审
doi:10.1016/s0378-5955(00)00136-2
更新日期:2000-09-01 00:00:00
abstract::Although gap junctions occur in auditory and vestibular systems, their function is unclear. Here we present evidence for gap junctional communication in transmitting mechanosensory signals in a sea anemone model system. Hair bundles on anemone tentacles are vibration-sensitive mechanoreceptors that regulate discharge ...
journal_title:Hearing research
pub_type: 杂志文章
doi:10.1016/s0378-5955(00)00047-2
更新日期:2000-06-01 00:00:00
abstract::The present study presents an atlas of the effects of 10 kHz tone exposures of different levels and durations on cochlear hair cells and their stereocilia in the Syrian golden hamster. Animals were sound exposed while under anesthesia. The exposure conditions were varied over an intensity range of 90-129 dB SPL; at th...
journal_title:Hearing research
pub_type: 杂志文章
doi:10.1016/0378-5955(92)90022-f
更新日期:1992-07-01 00:00:00
abstract::The olivocochlear bundle was sectioned at the floor of the fourth ventricle in a series of cats. From three to thirty weeks post-operatively, recordings were made from single auditory-nerve fibers. Tuning curves, spontaneous discharge rates, and rate-level functions for tones at the characteristic frequency were measu...
journal_title:Hearing research
pub_type: 杂志文章
doi:10.1016/0378-5955(90)90105-x
更新日期:1990-11-01 00:00:00
abstract::Two methods were used to extract frequency specific information from the gross d.c. cochlear potential, the summating potential (SP). The first approach was to derive SP tuning curves using a two-tone simultaneous masking procedure; the second to obtain SP iso-response functions. The influence of various parameter cha...
journal_title:Hearing research
pub_type: 杂志文章
doi:10.1016/0378-5955(84)90009-1
更新日期:1984-11-01 00:00:00
abstract::Older adults are often reported in the literature to have greater difficulty than younger adults understanding speech in noise [Helfer and Wilber (1988). J. Acoust. Soc. Am, 859-893]. The poorer performance of older adults has been attributed to a general deterioration of cognitive processing, deterioration of cochlea...
journal_title:Hearing research
pub_type: 杂志文章
doi:10.1016/j.heares.2014.09.001
更新日期:2014-11-01 00:00:00
abstract::Unlike the organ of Corti in mammals, the avian basilar papilla has no distinct populations of hair cells. Instead, there is a continuous change between the extreme forms (tall hair cells = THC, and short hair cells = SHC). The hair-cell innervation pattern is complicated, there being no simple gradient between THC (m...
journal_title:Hearing research
pub_type: 杂志文章
doi:10.1016/0378-5955(92)90048-r
更新日期:1992-08-01 00:00:00
abstract::Gamma-aminobutyric acid (GABA)-like immunoreactivity in the chick basilar papilla and lagenar macula was studied by using anti-serum against GABA coupled with glutaraldehyde to bovine serum albumin. GABA-like immunoreactivity was localized selectively in the cytoplasm of both tall and short hair cells in the basilar p...
journal_title:Hearing research
pub_type: 杂志文章
doi:10.1016/0378-5955(87)90178-x
更新日期:1987-01-01 00:00:00
abstract::Sharp spatial selectivity is critical to auditory performance, particularly in pitch-related tasks. Most contemporary cochlear implants have employed monopolar stimulation that produces broad electric fields, which presumably contribute to poor pitch and pitch-related performance by implant users. Bipolar or tripolar ...
journal_title:Hearing research
pub_type: 杂志文章
doi:10.1016/j.heares.2011.11.005
更新日期:2012-01-01 00:00:00
abstract::Normal-hearing listeners can perceptually segregate concurrent sound sources, but listeners with significant hearing loss or who wear a cochlear implant (CI) lag behind in this ability. Perceptual grouping mechanisms are essential to segregate concurrent sound sources and affect comodulation masking release (CMR). Thu...
journal_title:Hearing research
pub_type: 杂志文章
doi:10.1016/j.heares.2012.11.023
更新日期:2013-02-01 00:00:00
abstract::The inner ear contains receptor cells that oscillate spontaneously, generating waves that propagate backward in the cochlea, ultimately causing sound to be radiated into the ear canal--the spontaneous otoacoustic emissions (SOAEs). Except in rare conditions, these internally generated signals appear to go unheard. The...
journal_title:Hearing research
pub_type: 杂志文章
doi:10.1016/s0378-5955(02)00388-x
更新日期:2002-09-01 00:00:00
abstract::Application of dexamethasone to the round window has been shown to ameliorate high frequency hearing loss resulting from the trauma of cochlear implantation in experimental animals, but elucidation of the factors influencing protection of the high frequencies has been confounded by the local trauma from electrode arra...
journal_title:Hearing research
pub_type: 杂志文章
doi:10.1016/j.heares.2010.03.006
更新日期:2010-06-14 00:00:00
abstract::We performed transient evoked otoacoustic emission (TEOAE) measurements on 29 ears of myasthenia gravis (MG) patients. The purpose of the study was to support the role of acetylcholine (ACh) in the efferent innervation of cochlear outer hair cells (OHCs). Another aim was to establish additional diagnostic tools for th...
journal_title:Hearing research
pub_type: 杂志文章
doi:10.1016/s0378-5955(98)00154-3
更新日期:1998-12-01 00:00:00