当前位置： SCI文献检索 > Scientific Reports期刊下所有文献 > Tmc2 expression partially restores auditory function in a mouse model of DFNB7/B11 deafness caused by loss of Tmc1 function.

Tmc2 expression partially restores auditory function in a mouse model of DFNB7/B11 deafness caused by loss of Tmc1 function.

Abstract：

:Mouse Tmc1 and Tmc2 are required for sensory transduction in cochlear and vestibular hair cells. Homozygous Tmc1∆/∆ mice are deaf, Tmc2∆/∆ mice have normal hearing, and double homozygous Tmc1∆/∆; Tmc2∆/∆ mice have deafness and profound vestibular dysfunction. These phenotypes are consistent with their different spatiotemporal expression patterns. Tmc1 expression is persistent in cochlear and vestibular hair cells, whereas Tmc2 expression is transient in cochlear hair cells but persistent in vestibular hair cells. On the basis of these findings, we hypothesized that persistent Tmc2 expression in mature cochlear hair cells could restore auditory function in Tmc1∆/∆ mice. To express Tmc2 in mature cochlear hair cells, we generated a transgenic mouse line, Tg[PTmc1::Tmc2], in which Tmc2 cDNA is expressed under the control of the Tmc1 promoter. The Tg[PTmc1::Tmc2] transgene slightly but significantly restored hearing in young Tmc1∆/∆ mice, though hearing thresholds were elevated with age. The elevation of hearing thresholds was associated with deterioration of sensory transduction in inner hair cells and loss of outer hair cell function. Although sensory transduction was retained in outer hair cells, their stereocilia eventually degenerated. These results indicate distinct roles and requirements for Tmc1 and Tmc2 in mature cochlear hair cells.

journal_name

Sci Rep

journal_title

Scientific reports

authors

Nakanishi H,Kurima K,Pan B,Wangemann P,Fitzgerald TS,Géléoc GS,Holt JR,Griffith AJ

doi

10.1038/s41598-018-29709-8

subject

Has Abstract

pub_date

2018-08-14 00:00:00

pages

12125

issue

1

issn

2045-2322

pii

10.1038/s41598-018-29709-8

journal_volume

8

pub_type

杂志文章

相关文献

Scientific Reports文献大全
  • Crystalline structure, electronic and lattice-dynamics properties of NbTe2.

    abstract：:Layered-structure materials are currently relevant given their quasi-2D nature. Knowledge of their physical properties is currently of major interest. Niobium ditelluride possesses a monoclinic layered-structure with a distortion in the tellurium planes. This structural complexity has hindered the determination of its...

    journal_title：Scientific reports

    pub_type： 杂志文章

    doi：10.1038/s41598-018-35308-4

    authors： Barajas-Aguilar AH,Irwin JC,Garay-Tapia AM,Schwarz T,Paraguay Delgado F,Brodersen PM,Prinja R,Kherani N,Jiménez Sandoval SJ

    更新日期：2018-11-19 00:00:00

  • HIV-1 Tat-induced diarrhea evokes an enteric glia-dependent neuroinflammatory response in the central nervous system.

    abstract：:Despite the effectiveness of combined anti-retroviral therapy, human immunodeficiency virus (HIV) infected-patients frequently report diarrhea and neuropsychological deficits. It is claimed that the viral HIV-1 Trans activating factor (HIV-1 Tat) protein is responsible for both diarrhea and neurotoxic effects, but the...

    journal_title：Scientific reports

    pub_type： 杂志文章

    doi：10.1038/s41598-017-05245-9

    authors： Esposito G,Capoccia E,Gigli S,Pesce M,Bruzzese E,D'Alessandro A,Cirillo C,di Cerbo A,Cuomo R,Seguella L,Steardo L,Sarnelli G

    更新日期：2017-08-10 00:00:00

  • Whole Mitogenomes Reveal the History of Swamp Buffalo: Initially Shaped by Glacial Periods and Eventually Modelled by Domestication.

    abstract：:The newly sequenced mitochondrial genomes of 107 Asian swamp buffalo (Bubalus bubalis carabensis) allowed the reconstruction of the matrilineal divergence since ~900 Kya. Phylogenetic trees and Bayesian skyline plots suggest a role of the glacial periods in the demographic history of swamp buffalo. The ancestral swamp...

    journal_title：Scientific reports

    pub_type： 杂志文章

    doi：10.1038/s41598-017-04830-2

    authors： Wang S,Chen N,Capodiferro MR,Zhang T,Lancioni H,Zhang H,Miao Y,Chanthakhoun V,Wanapat M,Yindee M,Zhang Y,Lu H,Caporali L,Dang R,Huang Y,Lan X,Plath M,Chen H,Lenstra JA,Achilli A,Lei C

    更新日期：2017-07-05 00:00:00

  • The m6A-methylase complex recruits TREX and regulates mRNA export.

    abstract：:N6-methyladenosine (m6A) is the most abundant internal modification of eukaryotic mRNA. This modification has previously been shown to alter the export kinetics for mRNAs though the molecular details surrounding this phenomenon remain poorly understood. Recruitment of the TREX mRNA export complex to mRNA is driven by ...

    journal_title：Scientific reports

    pub_type： 杂志文章

    doi：10.1038/s41598-018-32310-8

    authors： Lesbirel S,Viphakone N,Parker M,Parker J,Heath C,Sudbery I,Wilson SA

    更新日期：2018-09-14 00:00:00

  • A dynamic prediction model for prognosis of acute-on-chronic liver failure based on the trend of clinical indicators.

    abstract：:Acute-on-chronic liver failure (ACLF) is a dynamic syndrome, and sequential assessments can reflect its prognosis more accurately. Our aim was to build and validate a new scoring system to predict short-term prognosis using baseline and dynamic data in ACLF. We conducted a retrospective cohort analysis of patients wit...

    journal_title：Scientific reports

    pub_type： 杂志文章

    doi：10.1038/s41598-021-81431-0

    authors： Yu Z,Zhang Y,Cao Y,Xu M,You S,Chen Y,Zhu B,Kong M,Song F,Xin S,Duan Z,Han T

    更新日期：2021-01-19 00:00:00

  • Shape-modification of patterned nanoparticles by an ion beam treatment.

    abstract：:This paper evaluated a practical approach to the fabrication of arrays of non-spherical nanoparticles by colloidal etching without a mask involving exposure to a low energy ion beam. A spherical nanoparticle array was transferred using a soft nanolithography technique, which is a simple and effective pattern transfer ...

    journal_title：Scientific reports

    pub_type： 杂志文章

    doi：10.1038/srep08523

    authors： Heo KC,Gwag JS

    更新日期：2015-02-17 00:00:00

  • Entangled-photon decision maker.

    abstract：:The competitive multi-armed bandit (CMAB) problem is related to social issues such as maximizing total social benefits while preserving equality among individuals by overcoming conflicts between individual decisions, which could seriously decrease social benefits. The study described herein provides experimental evide...

    journal_title：Scientific reports

    pub_type： 杂志文章

    doi：10.1038/s41598-019-48647-7

    authors： Chauvet N,Jegouso D,Boulanger B,Saigo H,Okamura K,Hori H,Drezet A,Huant S,Bachelier G,Naruse M

    更新日期：2019-08-22 00:00:00

  • Trophic factor-induced activity 'signature' regulates the functional expression of postsynaptic excitatory acetylcholine receptors required for synaptogenesis.

    abstract：:Highly coordinated and coincidental patterns of activity-dependent mechanisms ("fire together wire together") are thought to serve as inductive signals during synaptogenesis, enabling neuronal pairing between specific sub-sets of excitatory partners. However, neither the nature of activity triggers, nor the "activity ...

    journal_title：Scientific reports

    pub_type： 杂志文章

    doi：10.1038/srep09523

    authors： Luk CC,Lee AJ,Wijdenes P,Zaidi W,Leung A,Wong NY,Andrews J,Syed NI

    更新日期：2015-04-01 00:00:00

  • Effect of in vitro storage duration on measured mechanical properties of brain tissue.

    abstract：:Accurate characterization of the mechanical properties of brain tissue is essential for understanding the mechanisms of traumatic brain injuries and developing protective gears or facilities. However, how storage conditions might affect the mechanical properties of brain tissue remains unclear. The objective of this s...

    journal_title：Scientific reports

    pub_type： 杂志文章

    doi：10.1038/s41598-018-19687-2

    authors： Zhang W,Liu LF,Xiong YJ,Liu YF,Yu SB,Wu CW,Guo W

    更新日期：2018-01-19 00:00:00

  • A Guide to Design Functional Molecular Liquids with Tailorable Properties using Pyrene-Fluorescence as a Probe.

    abstract：:Solvent-free, nonvolatile, room-temperature alkylated-π functional molecular liquids (FMLs) are rapidly emerging as a new generation of fluid matter. However, precision design to tune their physicochemical properties remains a serious challenge because the properties are governed by subtle π-π interactions among funct...

    journal_title：Scientific reports

    pub_type： 杂志文章

    doi：10.1038/s41598-017-03584-1

    authors： Lu F,Takaya T,Iwata K,Kawamura I,Saeki A,Ishii M,Nagura K,Nakanishi T

    更新日期：2017-06-13 00:00:00

  • A novel immune prognostic index for stratification of high-risk patients with early breast cancer.

    abstract：:The prognostic value of current multigene assays for breast cancer is limited to hormone receptor-positive, human epidermal growth factor receptor 2-negative early breast cancer. Despite the prognostic significance of immune response-related genes in breast cancer, immune gene signatures have not been incorporated int...

    journal_title：Scientific reports

    pub_type： 杂志文章

    doi：10.1038/s41598-020-80274-5

    authors： Lee H,Kwon MJ,Koo BM,Park HG,Han J,Shin YK

    更新日期：2021-01-08 00:00:00

  • Tilt-less 3-D electron imaging and reconstruction of complex curvilinear structures.

    abstract：:The ability to obtain three-dimensional (3-D) information about morphologies of nanostructures elucidates many interesting properties of materials in both physical and biological sciences. Here we demonstrate a novel method in scanning transmission electron microscopy (STEM) that gives a fast and reliable assessment o...

    journal_title：Scientific reports

    pub_type： 杂志文章

    doi：10.1038/s41598-017-07537-6

    authors： Oveisi E,Letouzey A,Alexander DTL,Jeangros Q,Schäublin R,Lucas G,Fua P,Hébert C

    更新日期：2017-09-06 00:00:00

  • Natural rubber as a renewable carbon source for mesoporous carbon/silica nanocomposites.

    abstract：:This study is the first report on the preparation of mesoporous carbon/silica (MCS) nanocomposites with tunable mesoporosity and hydrophobicity using natural rubber (NR) as a renewable and cheap carbon source. A series of mesoporous nanocomposites based on NR and hexagonal mesoporous silica (HMS) were prepared via an ...

    journal_title：Scientific reports

    pub_type： 杂志文章

    doi：10.1038/s41598-020-69963-3

    authors： Yousatit S,Pitayachinchot H,Wijitrat A,Chaowamalee S,Nuntang S,Soontaranon S,Rugmai S,Yokoi T,Ngamcharussrivichai C

    更新日期：2020-07-31 00:00:00

  • A computational guided, functional validation of a novel therapeutic antibody proposes Notch signaling as a clinical relevant and druggable target in glioma.

    abstract：:The Notch signaling network determines stemness in various tissues and targeting signaling activity in malignant brain cancers by gamma-secretase inhibitors (GSI) has shown promising preclinical success. However, the clinical translation remains challenging due to severe toxicity side effects and emergence of therapy ...

    journal_title：Scientific reports

    pub_type： 杂志文章

    doi：10.1038/s41598-020-72480-y

    authors： Herrera-Rios D,Li G,Khan D,Tsiampali J,Nickel AC,Aretz P,Hewera M,Suwala AK,Jiang T,Steiger HJ,Kamp MA,Muhammad S,Hänggi D,Maciaczyk J,Zhang W,Kahlert UD

    更新日期：2020-10-01 00:00:00

  • Toward a more accurate view of human B-cell repertoire by next-generation sequencing, unbiased repertoire capture and single-molecule barcoding.

    abstract：:B-cell repertoire analysis using next-generation sequencing has become a valuable tool for interrogating the genetic record of humoral response to infection. However, key obstacles such as low throughput, short read length, high error rate, and undetermined bias of multiplex PCR method have hindered broader applicatio...

    journal_title：Scientific reports

    pub_type： 杂志文章

    doi：10.1038/srep06778

    authors： He L,Sok D,Azadnia P,Hsueh J,Landais E,Simek M,Koff WC,Poignard P,Burton DR,Zhu J

    更新日期：2014-10-27 00:00:00

  • Figure-ground perception in the awake mouse and neuronal activity elicited by figure-ground stimuli in primary visual cortex.

    abstract：:Figure-ground segregation is the process by which the visual system identifies image elements of figures and segregates them from the background. Previous studies examined figure-ground segregation in the visual cortex of monkeys where figures elicit stronger neuronal responses than backgrounds. It was demonstrated in...

    journal_title：Scientific reports

    pub_type： 杂志文章

    doi：10.1038/s41598-018-36087-8

    authors： Schnabel UH,Bossens C,Lorteije JAM,Self MW,Op de Beeck H,Roelfsema PR

    更新日期：2018-12-12 00:00:00

  • Two-Dimensional Brain Microtubule Structures Behave as Memristive Devices.

    abstract：:Microtubules (MTs) are cytoskeletal structures that play a central role in a variety of cell functions including cell division and cargo transfer. MTs are also nonlinear electrical transmission lines that produce and conduct electrical oscillations elicited by changes in either electric field and/or ionic gradients. T...

    journal_title：Scientific reports

    pub_type： 杂志文章

    doi：10.1038/s41598-019-48677-1

    authors： Cantero MDR,Perez PL,Scarinci N,Cantiello HF

    更新日期：2019-08-27 00:00:00

  • Mangiferin from Pueraria tuberosa reduces inflammation via inactivation of NLRP3 inflammasome.

    abstract：:Recent reports have demonstrated the role of phyto-constituents in modulating inflammatory responses. Mangiferin isolated from Mangifera indica is known to induce potent anti-oxidative, anti-diabetic and anti-inflammatory activity. However, the molecular mechanism of its anti-inflammatory activity is not properly unde...

    journal_title：Scientific reports

    pub_type： 杂志文章

    doi：10.1038/srep42683

    authors： Bulugonda RK,Kumar KA,Gangappa D,Beeda H,Philip GH,Muralidhara Rao D,Faisal SM

    更新日期：2017-02-20 00:00:00

  • Hepatobiliary MRI: Signal intensity based assessment of liver function correlated to 13C-Methacetin breath test.

    abstract：:Gadoxetic acid (Gd-EOB-DTPA) is a paramagnetic MRI contrast agent with raising popularity and has been used for evaluation of imaging-based liver function in recent years. In order to verify whether liver function as determined by real-time breath analysis using the intravenous administration of 13C-methacetin can be ...

    journal_title：Scientific reports

    pub_type： 杂志文章

    doi：10.1038/s41598-018-27401-5

    authors： Haimerl M,Probst U,Poelsterl S,Beyer L,Fellner C,Selgrad M,Hornung M,Stroszczynski C,Wiggermann P

    更新日期：2018-06-13 00:00:00

  • Gradient control of the adhesive force between Ti/TiO2 nanotubular arrays fabricated by anodization.

    abstract：:The poor control of the adhesion of TiO2 nanotubes (TNTs) layers to a non-anodized titanium (Ti) substrate has limited their widespread application, because the stripping mechanism has not yet been revealed. Here, we report a novel method to control the detachment of TNTs by post-treatment of the as-fabricated samples...

    journal_title：Scientific reports

    pub_type： 杂志文章

    doi：10.1038/srep07178

    authors： Zhao M,Li J,Li Y,Wang J,Zuo Y,Jiang J,Wang H

    更新日期：2014-11-24 00:00:00

  • Precisely dating the Frasnian-Famennian boundary: implications for the cause of the Late Devonian mass extinction.

    abstract：:The Frasnian-Famennian boundary records one of the most catastrophic mass extinctions of the Phanerozoic Eon. Several possible causes for this extinction have been suggested, including extra-terrestrial impacts and large-scale volcanism. However, linking the extinction with these potential causes is hindered by the la...

    journal_title：Scientific reports

    pub_type： 杂志文章

    doi：10.1038/s41598-018-27847-7

    authors： Percival LME,Davies JHFL,Schaltegger U,De Vleeschouwer D,Da Silva AC,Föllmi KB

    更新日期：2018-06-22 00:00:00

  • Probing conformational changes of monomeric transthyretin with second derivative fluorescence.

    abstract：:We have studied the intrinsic fluorescence spectra of a monomeric variant of human transthyretin (M-TTR), a protein involved in the transport of the thyroid hormone and retinol and associated with various forms of amyloidosis, extending our analysis to the second order derivative of the spectra. This procedure allowed...

    journal_title：Scientific reports

    pub_type： 杂志文章

    doi：10.1038/s41598-019-47230-4

    authors： Jazaj D,Ghadami SA,Bemporad F,Chiti F

    更新日期：2019-07-29 00:00:00

  • U-Th dated speleothem recorded geomagnetic excursions in the Lower Brunhes.

    abstract：:The study of geomagnetic excursions is key for understanding the behavior of the magnetic field of the Earth. In this paper, we present the geomagnetic record in a 2.29-m-long continuous core sampled in a flowstone in Liguria (Italy) and dated to the Lower Brunhes. The cored flowstone developed from Marine Isotopic St...

    journal_title：Scientific reports

    pub_type： 杂志文章

    doi：10.1038/s41598-018-38350-4

    authors： Pozzi JP,Rousseau L,Falguères C,Mahieux G,Deschamps P,Shao Q,Kachi D,Bahain JJ,Tozzi C

    更新日期：2019-02-04 00:00:00

  • The 5'UTR in human adenoviruses: leader diversity in late gene expression.

    abstract：:Human adenoviruses (HAdVs) shut down host cellular cap-dependent mRNA translation while initiating the translation of viral late mRNAs in a cap-independent manner. HAdV 5' untranslated regions (5'UTRs) are crucial for cap-independent initiation, and influence mRNA localization and stability. However, HAdV translationa...

    journal_title：Scientific reports

    pub_type： 杂志文章

    doi：10.1038/s41598-017-00747-y

    authors： Ramke M,Lee JY,Dyer DW,Seto D,Rajaiya J,Chodosh J

    更新日期：2017-04-04 00:00:00

  • Assessment of Risk and Resilience of Terrestrial Ecosystem Productivity under the Influence of Extreme Climatic Conditions over India.

    abstract：:Analysing the link between terrestrial ecosystem productivity (i.e., Net Primary Productivity: NPP) and extreme climate conditions is vital in the context of increasing threats due to climate change. To reveal the impact of changing extreme conditions on NPP, a copula-based probabilistic model was developed, and the s...

    journal_title：Scientific reports

    pub_type： 杂志文章

    doi：10.1038/s41598-019-55067-0

    authors： Jha S,Das J,Goyal MK

    更新日期：2019-12-12 00:00:00

  • Virtual screening of anti-HIV1 compounds against SARS-CoV-2: machine learning modeling, chemoinformatics and molecular dynamics simulation based analysis.

    abstract：:COVID-19 caused by the SARS-CoV-2 is a current global challenge and urgent discovery of potential drugs to combat this pandemic is a need of the hour. 3-chymotrypsin-like cysteine protease (3CLpro) enzyme is the vital molecular target against the SARS-CoV-2. Therefore, in the present study, 1528 anti-HIV1compounds wer...

    journal_title：Scientific reports

    pub_type： 杂志文章

    doi：10.1038/s41598-020-77524-x

    authors： Nand M,Maiti P,Joshi T,Chandra S,Pande V,Kuniyal JC,Ramakrishnan MA

    更新日期：2020-11-23 00:00:00

  • High genetic burden of type 2 diabetes can promote the high prevalence of disease: a longitudinal cohort study in Iran.

    abstract：:Type 2 diabetes (T2D) is emerging as one of the serious public health issues in both developed and developing counties. Here, we surveyed the worldwide population differentiation in T2D-associated variants and assessed the genetic burden of the disease in an ongoing Tehran Cardio-Metabolic Genetic Study (TCGS) cohort ...

    journal_title：Scientific reports

    pub_type： 杂志文章

    doi：10.1038/s41598-020-70725-4

    authors： Moazzam-Jazi M,Najd Hassan Bonab L,Zahedi AS,Daneshpour MS

    更新日期：2020-08-19 00:00:00

  • Primary rat LSECs preserve their characteristic phenotype after cryopreservation.

    abstract：:Liver disease is a leading cause of morbidity and mortality worldwide. Recently, the liver non-parenchymal cells have gained increasing attention for their potential role in the development of liver disease. Liver sinusoidal endothelial cells (LSECs), a specialized type of endothelial cells that have unique morphology...

    journal_title：Scientific reports

    pub_type： 杂志文章

    doi：10.1038/s41598-018-32103-z

    authors： Mönkemöller V,Mao H,Hübner W,Dumitriu G,Heimann P,Levy G,Huser T,Kaltschmidt B,Kaltschmidt C,Øie CI

    更新日期：2018-10-02 00:00:00

  • Identification of somatic alterations in lipoma using whole exome sequencing.

    abstract：:Lipomas are benign fatty tumors with a high prevalence rate, mostly found in adults but have a good prognosis. Until now, reason for lipoma occurrence not been identified. We performed whole exome sequencing to define the mutational spectrum in ten lipoma patients along with their matching control samples. We presente...

    journal_title：Scientific reports

    pub_type： 杂志文章

    doi：10.1038/s41598-019-50805-w

    authors： Kanojia D,Dakle P,Mayakonda A,Parameswaran R,Puhaindran ME,Min VLK,Madan V,Koeffler P

    更新日期：2019-10-07 00:00:00

  • Peripapillary microvascular changes in patients with systemic hypertension: An optical coherence tomography angiography study.

    abstract：:The purpose of this study was to investigate changes in peripapillary microvasculature using optical coherence tomography angiography (OCTA) in systemic hypertension (HTN) patients. This was a cross-sectional study. Based on the duration of HTN, seventy-eight HTN patients were divided into two groups. (HTN group 1: <1...

    journal_title：Scientific reports

    pub_type： 杂志文章

    doi：10.1038/s41598-020-63603-6

    authors： Shin YI,Nam KY,Lee WH,Ryu CK,Lim HB,Jo YJ,Kim JY

    更新日期：2020-04-16 00:00:00