{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,27]],"date-time":"2026-06-27T05:43:46Z","timestamp":1782539026973,"version":"3.54.5"},"reference-count":76,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2021,10,21]],"date-time":"2021-10-21T00:00:00Z","timestamp":1634774400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100012166","name":"National Key Research and Development Program of China","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100012166","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100019492","name":"National Natural Science Foundation of China-China Academy of General Technology Joint Fund for Basic Research","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100019492","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100012166","name":"National Key Research and Development Program of China","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100012166","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100019492","name":"National Natural Science Foundation of China-China Academy of General Technology Joint Fund for Basic Research","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100019492","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004826","name":"Natural Science Foundation of Beijing Municipality","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100004826","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Cell Dev. Biol."],"abstract":"<jats:p><jats:italic>ATP6V1B2<\/jats:italic> encodes the V1B2 subunit in V-ATPase, a proton pump responsible for the acidification of lysosomes. Mutations in this gene cause DDOD syndrome, DOORS syndrome, and Zimmermann\u2013Laband syndrome, which share overlapping feature of congenital sensorineural deafness, onychodystrophy, and different extents of intellectual disability without or with epilepsy. However, the underlying mechanisms remain unclear. To investigate the pathological role of mutant <jats:italic>ATP6V1B2<\/jats:italic> in the auditory system, we evaluated auditory brainstem response, distortion product otoacoustic emissions, in a transgenic line of mice carrying c.1516 C &amp;gt; T (p.Arg506<jats:sup>\u2217<\/jats:sup>) in <jats:italic>Atp6v1b2, Atp6v1b2<\/jats:italic><jats:sup><jats:italic>Arg506*\/Arg506*<\/jats:italic><\/jats:sup>. To explore the pathogenic mechanism of neurodegeneration in the auditory pathway, immunostaining, western blotting, and RNAscope analyses were performed in <jats:italic>Atp6v1b2<jats:sup>Arg506*\/Arg506*<\/jats:sup><\/jats:italic> mice. The <jats:italic>Atp6v1b2<jats:sup>Arg506*\/Arg506*<\/jats:sup><\/jats:italic> mice showed hidden hearing loss (HHL) at early stages and developed late-onset hearing loss. We observed increased transcription of <jats:italic>Atp6v1b1<\/jats:italic> in hair cells of <jats:italic>Atp6v1b2<jats:sup>Arg506*\/Arg506*<\/jats:sup><\/jats:italic> mice and inferred that <jats:italic>Atp6v1b1<\/jats:italic> compensated for the <jats:italic>Atp6v1b2<\/jats:italic> dysfunction by increasing its own transcription level. Genetic compensation in hair cells explains the milder hearing impairment in <jats:italic>Atp6v1b2<jats:sup>Arg506*\/Arg506*<\/jats:sup><\/jats:italic> mice. Apoptosis activated by lysosomal dysfunction and the subsequent blockade of autophagic flux induced the degeneration of spiral ganglion neurons and further impaired the hearing. Intraperitoneal administration of the apoptosis inhibitor, BIP-V5, improved both phenotypical and pathological outcomes in two live mutant mice. Based on the pathogenesis underlying hearing loss in <jats:italic>Atp6v1b2<\/jats:italic>-related syndromes, systemic drug administration to inhibit apoptosis might be an option for restoring the function of spiral ganglion neurons and promoting hearing, which provides a direction for future treatment.<\/jats:p>","DOI":"10.3389\/fcell.2021.742714","type":"journal-article","created":{"date-parts":[[2021,10,21]],"date-time":"2021-10-21T16:38:02Z","timestamp":1634834282000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":13,"title":["Syndromic Deafness Gene ATP6V1B2 Controls Degeneration of Spiral Ganglion Neurons Through Modulating Proton Flux"],"prefix":"10.3389","volume":"9","author":[{"given":"Shiwei","family":"Qiu","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Weihao","family":"Zhao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xue","family":"Gao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dapeng","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Weiqian","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bo","family":"Gao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Weiju","family":"Han","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shiming","family":"Yang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pu","family":"Dai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peng","family":"Cao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yongyi","family":"Yuan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2021,10,21]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"39","DOI":"10.1159\/000502632","article-title":"Resveratrol pretreatment ameliorates p53-bax axis and augments the survival biomarker B-cell lymphoma 2 modulated by paracetamol overdose in a rat model of acute liver injury.","volume":"105","author":"Al Humayed","year":"2020","journal-title":"Pharmacology"},{"key":"B2","doi-asserted-by":"publisher","first-page":"1674","DOI":"10.1111\/j.1440-1746.2005.04057.x","article-title":"Bax and Bcl-2 protein expression in gastric precancerous lesions: immunohistochemical study.","volume":"20","author":"Anagnostopoulos","year":"2005","journal-title":"J. Gastroenterol. Hepatol."},{"key":"B3","doi-asserted-by":"publisher","first-page":"149","DOI":"10.1038\/s41436-020-00950-9","article-title":"DOORS syndrome and a recurrent truncating ATP6V1B2 variant.","volume":"23","author":"Beauregard-Lacroix","year":"2020","journal-title":"Genet. Med."},{"key":"B4","doi-asserted-by":"crossref","first-page":"577","DOI":"10.1242\/jeb.02014","article-title":"The V-type H+ ATPase: molecular structure and function, physiological roles and regulation.","volume":"209","author":"Beyenbach","year":"2006","journal-title":"J. Exp. Biol."},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.3389\/fnsys.2014.00026","article-title":"Cochlear neuropathy and the coding of supra-threshold sound.","volume":"8","author":"Bharadwaj","year":"2014","journal-title":"Front. Syst. Neurosci."},{"key":"B6","doi-asserted-by":"crossref","first-page":"3702","DOI":"10.2174\/0929867325666180226094351","article-title":"Role of autophagy in Parkinson\u2019s disease.","volume":"26","author":"Cerri","year":"2019","journal-title":"Curr. Med. Chem."},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1016\/j.celrep.2021.109016","article-title":"Generation of mature and functional hair cells by co-expression of Gfi1, Pou4f3, and Atoh1 in the postnatal mouse cochlea.","volume":"35","author":"Chen","year":"2021","journal-title":"Cell Rep."},{"key":"B8","doi-asserted-by":"publisher","first-page":"324","DOI":"10.1016\/j.jgg.2021.03.010","article-title":"Disruption of the autism-related gene Pak1 causes stereocilia disorganization, hair cell loss, and deafness in mice.","volume":"48","author":"Cheng","year":"2021","journal-title":"J. Genet. Genomics"},{"key":"B9","doi-asserted-by":"crossref","DOI":"10.1186\/s13287-019-1437-0","article-title":"Age-related transcriptome changes in Sox2+ supporting cells in the mouse cochlea.","volume":"10","author":"Cheng","year":"2019","journal-title":"Stem Cell Res. Ther."},{"key":"B10","doi-asserted-by":"publisher","first-page":"75","DOI":"10.1016\/j.arr.2016.05.004","article-title":"Disorders of lysosomal acidification\u2013the emerging role of v-ATPase in aging and neurodegenerative disease.","volume":"32","author":"Colacurcio","year":"2016","journal-title":"Ageing Res. Rev."},{"key":"B11","first-page":"113","article-title":"[The lysosomes: a novel group of cytoplasmic granules].","volume":"49","author":"De Duve","year":"1957","journal-title":"J. Physiol. (Paris)"},{"key":"B12","doi-asserted-by":"publisher","DOI":"10.3389\/fcell.2020.614954","article-title":"The role of FoxG1 in the inner ear.","volume":"8","author":"Ding","year":"2020","journal-title":"Front. Cell Dev. Biol"},{"key":"B13","doi-asserted-by":"publisher","first-page":"193","DOI":"10.1038\/s41586-019-1064-z","article-title":"Genetic compensation triggered by mutant mRNA degradation.","volume":"568","author":"El-Brolosy","year":"2019","journal-title":"Nature"},{"key":"B14","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1159\/000499582","article-title":"Age-dependent changes in the Cochlea.","volume":"66","author":"Fischer","year":"2019","journal-title":"Gerontology"},{"key":"B15","doi-asserted-by":"crossref","first-page":"4376","DOI":"10.1007\/s12035-021-02422-w","article-title":"Deficiency of Klc2 induces low-frequency sensorineural hearing loss in C57BL\/6 J mice and human.","volume":"58","author":"Fu","year":"2021","journal-title":"Mol. Neurobiol."},{"key":"B16","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1038\/cdd.2011.96","article-title":"Molecular definitions of cell death subroutines: recommendations of the nomenclature committee on cell death 2012.","volume":"19","author":"Galluzzi","year":"2012","journal-title":"Cell Death Differ."},{"key":"B17","doi-asserted-by":"publisher","DOI":"10.3389\/fncel.2019.00590","article-title":"Blebbistatin inhibits neomycin-induced apoptosis in hair cell-like HEI-OC-1 cells and in cochlear hair cells.","volume":"13","author":"Gao","year":"2019","journal-title":"Front. Cell. Neurosci."},{"key":"B18","doi-asserted-by":"crossref","first-page":"6735","DOI":"10.1021\/acsbiomaterials.9b01265","article-title":"Development and application of cochlear implant-based electric-acoustic stimulation of spiral ganglion neurons.","volume":"5","author":"Guo","year":"2019","journal-title":"ACS Biomater. Sci. Eng."},{"key":"B19","doi-asserted-by":"crossref","DOI":"10.1016\/j.actbio.2020.12.035","article-title":"2D Ti3C2TxMXene couples electrical stimulation to promote proliferation and neural differentiation of neural stem cells.","author":"Guo","year":"2020","journal-title":"Acta Biomater."},{"key":"B20","doi-asserted-by":"publisher","first-page":"193","DOI":"10.1016\/j.biomaterials.2016.08.019","article-title":"Accelerating bioelectric functional development of neural stem cells by graphene coupling: implications for neural interfacing with conductive materials.","volume":"106","author":"Guo","year":"2016","journal-title":"Biomaterials"},{"key":"B21","doi-asserted-by":"publisher","DOI":"10.1016\/j.bios.2020.112073","article-title":"Isolation and analysis of extracellular vesicles in a Morpho butterfly wing-integrated microvortex biochip.","volume":"154","author":"Han","year":"2020","journal-title":"Biosens. Bioelectron."},{"key":"B22","doi-asserted-by":"publisher","first-page":"1884","DOI":"10.1080\/15548627.2017.1359449","article-title":"Autophagy protects auditory hair cells against neomycin-induced damage.","volume":"13","author":"He","year":"2017","journal-title":"Autophagy"},{"key":"B23","first-page":"1","article-title":"FOXG1 promotes aging inner ear hair cell survival through activation of the autophagy pathway.","volume":"19","author":"He","year":"2021","journal-title":"Autophagy"},{"key":"B24","doi-asserted-by":"publisher","DOI":"10.1016\/j.redox.2019.101364","article-title":"The nuclear transcription factor FoxG1 affects the sensitivity of mimetic aging hair cells to inflammation by regulating autophagy pathways.","volume":"28","author":"He","year":"2020","journal-title":"Redox Biol."},{"key":"B25","doi-asserted-by":"publisher","first-page":"261","DOI":"10.1038\/nature11654","article-title":"An early age increase in vacuolar pH limits mitochondrial function and lifespan in yeast.","volume":"492","author":"Hughes","year":"2012","journal-title":"Nature"},{"key":"B26","doi-asserted-by":"publisher","first-page":"3952","DOI":"10.3390\/ijms15033952","article-title":"Mitochondria in the center of human eosinophil apoptosis and survival.","volume":"15","author":"Ilmarinen","year":"2014","journal-title":"Int. J. Mol. Sci."},{"key":"B27","doi-asserted-by":"publisher","DOI":"10.3389\/fbioe.2020.00525","article-title":"The roles of exosomes in visual and auditory systems.","volume":"8","author":"Jiang","year":"2020","journal-title":"Front. Bioeng. Biotechnol."},{"key":"B28","doi-asserted-by":"publisher","first-page":"1021","DOI":"10.1067\/msy.2002.128616","article-title":"Apoptosis regulating genes, bcl-2 and bax, and human telomerase reverse transcriptase messenger RNA expression in adrenal tumors: possible diagnostic and prognostic importance.","volume":"132","author":"Kanauchi","year":"2002","journal-title":"Surgery"},{"key":"B29","doi-asserted-by":"crossref","DOI":"10.1038\/srep14027","article-title":"Loss of G2 subunit of vacuolar-type proton transporting ATPase leads to G1 subunit upregulation in the brain.","volume":"5","author":"Kawamura","year":"2015","journal-title":"Sci. Rep."},{"key":"B30","doi-asserted-by":"publisher","first-page":"661","DOI":"10.1038\/ng.3282","article-title":"Mutations in KCNH1 and ATP6V1B2 cause zimmermann-laband syndrome.","volume":"47","author":"Kortum","year":"2015","journal-title":"Nat. Genet."},{"key":"B31","doi-asserted-by":"publisher","first-page":"99","DOI":"10.1152\/physrev.00013.2006","article-title":"Mitochondrial membrane permeabilization in cell death.","volume":"87","author":"Kroemer","year":"2007","journal-title":"Physiol. Rev."},{"key":"B32","doi-asserted-by":"publisher","first-page":"14077","DOI":"10.1523\/jneurosci.2845-09.2009","article-title":"Adding insult to injury: cochlear nerve degeneration after \u201ctemporary\u201d noise-induced hearing loss.","volume":"29","author":"Kujawa","year":"2009","journal-title":"J. Neurosci."},{"key":"B33","doi-asserted-by":"publisher","first-page":"138","DOI":"10.1016\/j.heares.2017.01.003","article-title":"Cochlear synaptopathy in acquired sensorineural hearing loss: manifestations and mechanisms.","volume":"349","author":"Liberman","year":"2017","journal-title":"Hear. Res."},{"key":"B34","doi-asserted-by":"publisher","DOI":"10.1016\/j.neulet.2019.05.022","article-title":"Functional alteration of ribbon synapses in inner hair cells by noise exposure causing hidden hearing loss.","volume":"707","author":"Liu","year":"2019","journal-title":"Neurosci. Lett."},{"key":"B35","doi-asserted-by":"publisher","first-page":"1680","DOI":"10.1007\/s12035-014-8951-y","article-title":"Spontaneous and partial repair of ribbon synapse in cochlear inner hair cells after ototoxic withdrawal.","volume":"52","author":"Liu","year":"2015","journal-title":"Mol. Neurobiol."},{"key":"B36","doi-asserted-by":"publisher","DOI":"10.1038\/cddis.2016.35","article-title":"Wnt activation protects against neomycin-induced hair cell damage in the mouse cochlea.","volume":"7","author":"Liu","year":"2016","journal-title":"Cell Death Dis."},{"key":"B37","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1080\/15548627.2021.1905466","article-title":"PRDX1 activates autophagy via the PTEN-AKT signaling pathway to protect against cisplatin-induced spiral ganglion neuron damage.","volume":"12","author":"Liu","year":"2021","journal-title":"Autophagy"},{"key":"B38","doi-asserted-by":"publisher","first-page":"1389","DOI":"10.1089\/ars.2017.7288","article-title":"Wnt signaling activates TP53-induced glycolysis and apoptosis regulator and protects against cisplatin-induced spiral ganglion neuron damage in the mouse cochlea.","volume":"30","author":"Liu","year":"2019","journal-title":"Antioxid. Redox Signal."},{"key":"B39","doi-asserted-by":"publisher","DOI":"10.1126\/sciadv.aav7803","article-title":"Critical role of spectrin in hearing development and deafness.","volume":"5","author":"Liu","year":"2019","journal-title":"Sci. Adv."},{"key":"B40","doi-asserted-by":"publisher","DOI":"10.3389\/fcell.2021.630361","article-title":"Deletion of Kcnj16 in mice does not alter auditory function.","volume":"9","author":"Lv","year":"2021","journal-title":"Front. Cell Dev. Biol."},{"key":"B41","doi-asserted-by":"publisher","first-page":"1244","DOI":"10.1016\/j.cellsig.2011.03.003","article-title":"Structural bases of physiological functions and roles of the vacuolar H(+)-ATPase.","volume":"23","author":"Ma","year":"2011","journal-title":"Cell. Signal."},{"key":"B42","doi-asserted-by":"publisher","first-page":"259","DOI":"10.1038\/s41586-019-1057-y","article-title":"PTC-bearing mRNA elicits a genetic compensation response via Upf3a and COMPASS components.","volume":"568","author":"Ma","year":"2019","journal-title":"Nature"},{"key":"B43","doi-asserted-by":"crossref","DOI":"10.1038\/ncomms8007","article-title":"Autophagosome-lysosome fusion is independent of V-ATPase-mediated acidification.","volume":"6","author":"Mauvezin","year":"2015","journal-title":"Nat. Commun."},{"key":"B44","doi-asserted-by":"publisher","first-page":"3755","DOI":"10.1523\/jneurosci.4460-15.2016","article-title":"Auditory brainstem response latency in noise as a marker of cochlear synaptopathy.","volume":"36","author":"Mehraei","year":"2016","journal-title":"J. Neurosci."},{"key":"B45","doi-asserted-by":"publisher","first-page":"376","DOI":"10.1002\/ccr3.761","article-title":"Dominant deafness-onychodystrophy syndrome caused by an ATP6V1B2 mutation.","volume":"5","author":"Menendez","year":"2017","journal-title":"Clin. Case Rep."},{"key":"B46","doi-asserted-by":"publisher","first-page":"69","DOI":"10.1146\/annurev-physiol-012110-142317","article-title":"Lysosomal acidification mechanisms.","volume":"74","author":"Mindell","year":"2012","journal-title":"Annu. Rev. Physiol."},{"key":"B47","doi-asserted-by":"crossref","DOI":"10.1155\/2014\/913071","article-title":"Linking peroxiredoxin and vacuolar-ATPase functions in calorie restriction-mediated life span extension.","volume":"2014","author":"Molin","year":"2014","journal-title":"Int. J. Cell Biol."},{"key":"B48","doi-asserted-by":"crossref","first-page":"F1915","DOI":"10.1152\/ajprenal.00160.2007","article-title":"Compensatory membrane expression of the V-ATPase B2 subunit isoform in renal medullary intercalated cells of B1-deficient mice.","volume":"293","author":"Paunescu","year":"2007","journal-title":"Am. J. Physiol. Renal Physiol."},{"key":"B49","doi-asserted-by":"publisher","first-page":"363","DOI":"10.1007\/s00401-014-1379-7","article-title":"Early etiology of Alzheimer\u2019s disease: tipping the balance toward autophagy or endosomal dysfunction?","volume":"129","author":"Peric","year":"2015","journal-title":"Acta Neuropathol."},{"key":"B50","doi-asserted-by":"crossref","DOI":"10.1038\/s41421-018-0076-4","article-title":"A cytoskeleton structure revealed by super-resolution fluorescence imaging in inner ear hair cells.","volume":"5","author":"Qi","year":"2019","journal-title":"Cell Discov."},{"key":"B51","first-page":"130","article-title":"Espin distribution as revealed by super-resolution microscopy of stereocilia.","volume":"12","author":"Qi","year":"2020","journal-title":"Am. J. Transl. Res."},{"key":"B52","doi-asserted-by":"publisher","first-page":"18804","DOI":"10.18632\/aging.103840","article-title":"The slc4a2b gene is required for hair cell development in zebrafish.","volume":"12","author":"Qian","year":"2020","journal-title":"Aging (Albany NY)"},{"key":"B53","doi-asserted-by":"publisher","DOI":"10.1101\/cshperspect.a020495","article-title":"The nodes of ranvier: molecular assembly and maintenance.","volume":"8","author":"Rasband","year":"2015","journal-title":"Cold Spring Harb. Perspect. Biol."},{"key":"B54","doi-asserted-by":"publisher","first-page":"230","DOI":"10.1038\/nature14580","article-title":"Genetic compensation induced by deleterious mutations but not gene knockdowns.","volume":"524","author":"Rossi","year":"2015","journal-title":"Nature"},{"key":"B55","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pgen.1004347","article-title":"Lifespan extension by methionine restriction requires autophagy-dependent vacuolar acidification.","volume":"10","author":"Ruckenstuhl","year":"2014","journal-title":"PLoS Genet."},{"key":"B56","doi-asserted-by":"publisher","first-page":"1025","DOI":"10.1371\/journal.pgen.1004347","article-title":"Turn up the lysosome.","volume":"18","author":"Saftig","year":"2016","journal-title":"Nat. Cell Biol."},{"key":"B57","doi-asserted-by":"crossref","first-page":"1229","DOI":"10.1016\/j.cell.2018.07.007","article-title":"Sensory Neuron diversity in the inner ear is shaped by activity.","volume":"174","author":"Shrestha","year":"2018","journal-title":"Cell"},{"key":"B58","doi-asserted-by":"publisher","first-page":"114","DOI":"10.1097\/aud.0000000000000734","article-title":"Genetic inheritance of late-onset, down-sloping hearing loss and its implications for auditory rehabilitation.","volume":"41","author":"Song","year":"2020","journal-title":"Ear Hear."},{"key":"B59","doi-asserted-by":"crossref","first-page":"1247","DOI":"10.1016\/j.cell.2018.07.008","article-title":"Hair cell mechanotransduction regulates spontaneous activity and spiral ganglion subtype specification in the auditory system.","volume":"174","author":"Sun","year":"2018","journal-title":"Cell"},{"key":"B60","doi-asserted-by":"crossref","DOI":"10.1038\/s41467-019-11687-8","article-title":"AAV-ie enables safe and efficient gene transfer to inner ear cells.","volume":"10","author":"Tan","year":"2019","journal-title":"Nat. Commun."},{"key":"B61","doi-asserted-by":"publisher","first-page":"3722","DOI":"10.1093\/hmg\/ddx257","article-title":"Hearing loss without overt metabolic acidosis in ATP6V1B1 deficient MRL mice, a new genetic model for non-syndromic deafness with enlarged vestibular aqueducts.","volume":"26","author":"Tian","year":"2017","journal-title":"Hum. Mol. Genet."},{"key":"B62","doi-asserted-by":"publisher","first-page":"257","DOI":"10.1038\/ng848","article-title":"Beethoven, a mouse model for dominant, progressive hearing loss DFNA36.","volume":"30","author":"Vreugde","year":"2002","journal-title":"Nat. Genet."},{"key":"B63","doi-asserted-by":"crossref","DOI":"10.1038\/ncomms14487","article-title":"Transient auditory nerve demyelination as a new mechanism for hidden hearing loss.","volume":"8","author":"Wan","year":"2017","journal-title":"Nat. Commun."},{"key":"B64","doi-asserted-by":"publisher","DOI":"10.3389\/fnmol.2017.00401","article-title":"Loss of CIB2 causes profound hearing loss and abolishes mechanoelectrical transduction in mice.","volume":"10","author":"Wang","year":"2017","journal-title":"Front. Mol. Neurosci."},{"key":"B65","doi-asserted-by":"crossref","DOI":"10.1038\/s41598-017-03810-w","article-title":"Bone morphogenetic protein 4 promotes the survival and preserves the structure of flow-sorted Bhlhb5+ cochlear spiral ganglion neurons in vitro.","volume":"7","author":"Waqas","year":"2017","journal-title":"Sci. Rep."},{"key":"B66","doi-asserted-by":"publisher","first-page":"2070","DOI":"10.1007\/s12035-017-0471-0","article-title":"A three-dimensional culture system with matrigel promotes purified spiral ganglion neuron survival and function in vitro.","volume":"55","author":"Yan","year":"2018","journal-title":"Mol. Neurobiol."},{"key":"B67","doi-asserted-by":"publisher","first-page":"735","DOI":"10.1007\/s12264-021-00667-y","article-title":"Neuromodulation-based stem cell therapy in brain repair: recent advances and future perspectives.","volume":"37","author":"Yuan","year":"2021","journal-title":"Neurosci. Bull."},{"key":"B68","doi-asserted-by":"publisher","first-page":"1370","DOI":"10.1038\/cr.2014.77","article-title":"De novo mutation in ATP6V1B2 impairs lysosome acidification and causes dominant deafness-onychodystrophy syndrome.","volume":"24","author":"Yuan","year":"2014","journal-title":"Cell Res."},{"key":"B69","doi-asserted-by":"crossref","first-page":"355","DOI":"10.1038\/nature13992","article-title":"A comparative encyclopedia of DNA elements in the mouse genome.","volume":"515","author":"Yue","year":"2014","journal-title":"Nature"},{"key":"B70","doi-asserted-by":"publisher","DOI":"10.3389\/fgene.2021.625867","article-title":"Characterization of strip1 expression in mouse cochlear hair cells.","volume":"12","author":"Zhang","year":"2021","journal-title":"Front. Genet."},{"key":"B71","doi-asserted-by":"publisher","first-page":"1401","DOI":"10.1007\/s00018-019-03291-2","article-title":"Knockdown of Foxg1 in supporting cells increases the trans-differentiation of supporting cells into hair cells in the neonatal mouse cochlea.","volume":"77","author":"Zhang","year":"2020","journal-title":"Cell Mol. Life Sci."},{"key":"B72","doi-asserted-by":"publisher","first-page":"19834","DOI":"10.18632\/aging.104009","article-title":"Knockdown of Foxg1 in Sox9+ supporting cells increases the trans-differentiation of supporting cells into hair cells in the neonatal mouse utricle.","volume":"12","author":"Zhang","year":"2020","journal-title":"Aging (Albany NY)"},{"key":"B73","doi-asserted-by":"publisher","first-page":"408","DOI":"10.1016\/j.ebiom.2019.06.035","article-title":"A subunit of V-ATPases, ATP6V1B2, underlies the pathology of intellectual disability.","volume":"45","author":"Zhao","year":"2019","journal-title":"EBioMedicine"},{"key":"B74","doi-asserted-by":"publisher","DOI":"10.3389\/fcell.2020.00712","article-title":"Citicoline protects auditory hair cells against neomycin-induced damage.","volume":"8","author":"Zhong","year":"2020","journal-title":"Front. Cell Dev. Biol."},{"key":"B75","doi-asserted-by":"crossref","DOI":"10.1038\/s41419-020-03110-8","article-title":"Disruption of Atg7-dependent autophagy causes electromotility disturbances, outer hair cell loss, and deafness in mice.","volume":"11","author":"Zhou","year":"2020","journal-title":"Cell Death Dis."},{"key":"B76","doi-asserted-by":"publisher","first-page":"235","DOI":"10.1016\/j.acthis.2017.01.006","article-title":"Effect of dietary zinc on morphological characteristics and apoptosis related gene expression in the small intestine of Bama miniature pigs.","volume":"119","author":"Zhou","year":"2017","journal-title":"Acta Histochem."}],"container-title":["Frontiers in Cell and Developmental Biology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fcell.2021.742714\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,10,21]],"date-time":"2021-10-21T16:38:31Z","timestamp":1634834311000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fcell.2021.742714\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,10,21]]},"references-count":76,"alternative-id":["10.3389\/fcell.2021.742714"],"URL":"https:\/\/doi.org\/10.3389\/fcell.2021.742714","relation":{},"ISSN":["2296-634X"],"issn-type":[{"value":"2296-634X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,10,21]]},"article-number":"742714"}}