{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,4]],"date-time":"2026-09-04T17:53:19Z","timestamp":1788544399629,"version":"build-2803163510"},"reference-count":64,"publisher":"Elsevier BV","issue":"5","license":[{"start":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T00:00:00Z","timestamp":1777593600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T00:00:00Z","timestamp":1777593600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"}],"funder":[{"DOI":"10.13039\/501100012166","name":"National Key Research and Development Program of China","doi-asserted-by":"publisher","award":["2021YFA1101300"],"award-info":[{"award-number":["2021YFA1101300"]}],"id":[{"id":"10.13039\/501100012166","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100013290","name":"National Key Research and Development Program of China Stem Cell and Translational Research","doi-asserted-by":"publisher","award":["2021YFA1101800"],"award-info":[{"award-number":["2021YFA1101800"]}],"id":[{"id":"10.13039\/501100013290","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004608","name":"Jiangsu Province Natural Science Foundation","doi-asserted-by":"publisher","award":["BE2023653"],"award-info":[{"award-number":["BE2023653"]}],"id":[{"id":"10.13039\/501100004608","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004608","name":"Jiangsu Province Natural Science Foundation","doi-asserted-by":"publisher","award":["BK20230052"],"award-info":[{"award-number":["BK20230052"]}],"id":[{"id":"10.13039\/501100004608","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004608","name":"Jiangsu Province Natural Science Foundation","doi-asserted-by":"publisher","award":["BK20232007"],"award-info":[{"award-number":["BK20232007"]}],"id":[{"id":"10.13039\/501100004608","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["82192862"],"award-info":[{"award-number":["82192862"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["82330033"],"award-info":[{"award-number":["82330033"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["82171144"],"award-info":[{"award-number":["82171144"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["82371157"],"award-info":[{"award-number":["82371157"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["92149304"],"award-info":[{"award-number":["92149304"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["cell.com","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Molecular Therapy"],"published-print":{"date-parts":[[2026,5]]},"DOI":"10.1016\/j.ymthe.2026.01.030","type":"journal-article","created":{"date-parts":[[2026,1,29]],"date-time":"2026-01-29T15:43:37Z","timestamp":1769701417000},"page":"2801-2817","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":2,"title":["Dysregulation of Serpinb6a-Gch1 axis contributes to DFNB91 deafness that is amendable to gene therapies"],"prefix":"10.1016","volume":"34","author":[{"given":"Cheng","family":"Cheng","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Liyan","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jie","family":"Lu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fangzhi","family":"Tan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yideng","family":"Huang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Song","family":"Gao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Siyu","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yue","family":"Qiu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wenli","family":"Hao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yingyi","family":"Zhou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Junze","family":"Lu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xinya","family":"Ji","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ao","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xinru","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jinyi","family":"Fan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"He","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaoyun","family":"Qian","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jieyu","family":"Qi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xia","family":"Gao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3885-543X","authenticated-orcid":false,"given":"Renjie","family":"Chai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.ymthe.2026.01.030_bib1","unstructured":"Available from: https:\/\/www.who.int\/health-topics\/hearing-loss."},{"key":"10.1016\/j.ymthe.2026.01.030_bib2","doi-asserted-by":"crossref","first-page":"2195","DOI":"10.1001\/jama.2020.17647","article-title":"Hearing Loss in Children: A Review","volume":"324","author":"Lieu","year":"2020","journal-title":"JAMA"},{"key":"10.1016\/j.ymthe.2026.01.030_bib3","doi-asserted-by":"crossref","first-page":"2089","DOI":"10.1182\/blood.V93.6.2089.406k10_2089_2097","article-title":"The intracellular serpin proteinase inhibitor 6 is expressed in monocytes and granulocytes and is a potent inhibitor of the azurophilic granule protease, cathepsin G","volume":"93","author":"Scott","year":"1999","journal-title":"Blood"},{"key":"10.1016\/j.ymthe.2026.01.030_bib4","doi-asserted-by":"crossref","first-page":"435","DOI":"10.1093\/jb\/mvm156","article-title":"SerpinB6 is an inhibitor of kallikrein-8 in keratinocytes","volume":"142","author":"Scott","year":"2007","journal-title":"J. Biochem."},{"key":"10.1016\/j.ymthe.2026.01.030_bib5","doi-asserted-by":"crossref","first-page":"1108","DOI":"10.1016\/j.cell.2007.07.013","article-title":"An intracellular serpin regulates necrosis by inhibiting the induction and sequelae of lysosomal injury","volume":"130","author":"Luke","year":"2007","journal-title":"Cell"},{"key":"10.1016\/j.ymthe.2026.01.030_bib6","doi-asserted-by":"crossref","first-page":"1775","DOI":"10.14283\/jpad.2024.119","article-title":"Leveraging Diverse Regulated Cell Death Patterns to Identify Diagnosis Biomarkers for Alzheimer's Disease","volume":"11","author":"Ren","year":"2024","journal-title":"J. Prev. Alzheimers Dis."},{"key":"10.1016\/j.ymthe.2026.01.030_bib7","doi-asserted-by":"crossref","first-page":"3778","DOI":"10.1007\/s12035-022-02817-3","article-title":"Serpin Signatures in Prion and Alzheimer's Diseases","volume":"59","author":"Zattoni","year":"2022","journal-title":"Mol. Neurobiol."},{"key":"10.1016\/j.ymthe.2026.01.030_bib8","doi-asserted-by":"crossref","first-page":"16089","DOI":"10.1074\/jbc.270.27.16089","article-title":"Gene structure, chromosomal localization, and expression of the murine homologue of human proteinase inhibitor 6 (PI-6) suggests divergence of PI-6 from the ovalbumin serpins","volume":"270","author":"Sun","year":"1995","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.ymthe.2026.01.030_bib9","doi-asserted-by":"crossref","first-page":"797","DOI":"10.1016\/j.ajhg.2010.04.004","article-title":"A truncating mutation in SERPINB6 is associated with autosomal-recessive nonsyndromic sensorineural hearing loss","volume":"86","author":"Sirmaci","year":"2010","journal-title":"Am. J. Hum. Genet."},{"key":"10.1016\/j.ymthe.2026.01.030_bib10","doi-asserted-by":"crossref","DOI":"10.1155\/2021\/5584788","article-title":"Identification of Hearing Loss-Associated Variants of PTPRQ, MYO15A, and SERPINB6 in Pakistani Families","volume":"2021","author":"Mahmood","year":"2021","journal-title":"Biomed. Res. Int."},{"key":"10.1016\/j.ymthe.2026.01.030_bib11","doi-asserted-by":"crossref","first-page":"122","DOI":"10.1016\/j.jgg.2022.08.006","article-title":"Absence of Serpinb6a causes progressive hair cell apoptosis and hearing loss in mice","volume":"50","author":"Cheng","year":"2023","journal-title":"J. Genet. Genomics"},{"key":"10.1016\/j.ymthe.2026.01.030_bib12","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1016\/j.ajpath.2013.03.009","article-title":"Absence of SERPINB6A causes sensorineural hearing loss with multiple histopathologies in the mouse inner ear","volume":"183","author":"Tan","year":"2013","journal-title":"Am. J. Pathol."},{"key":"10.1016\/j.ymthe.2026.01.030_bib13","doi-asserted-by":"crossref","first-page":"1287","DOI":"10.1097\/WNR.0000000000001727","article-title":"Mice heterozygous for the Serpinb6a null mutation show deficits in central auditory function after acoustic trauma","volume":"32","author":"Tan","year":"2021","journal-title":"Neuroreport"},{"key":"10.1016\/j.ymthe.2026.01.030_bib14","doi-asserted-by":"crossref","first-page":"1638","DOI":"10.1111\/ejn.15011","article-title":"Increased susceptibility to acoustic trauma in a mouse model of non-syndromic sensorineural deafness, DFNB91","volume":"53","author":"Tan","year":"2021","journal-title":"Eur. J. Neurosci."},{"key":"10.1016\/j.ymthe.2026.01.030_bib15","doi-asserted-by":"crossref","first-page":"12065","DOI":"10.1111\/jcmm.15839","article-title":"Inhibition of ferroptosis protects House Ear Institute-Organ of Corti 1 cells and cochlear hair cells from cisplatin-induced ototoxicity","volume":"24","author":"Mei","year":"2020","journal-title":"J. Cell. Mol. Med."},{"key":"10.1016\/j.ymthe.2026.01.030_bib16","doi-asserted-by":"crossref","first-page":"133","DOI":"10.7150\/thno.37362","article-title":"Inhibition of Protein arginine methyltransferase 6 reduces reactive oxygen species production and attenuates aminoglycoside- and cisplatin-induced hair cell death","volume":"10","author":"He","year":"2020","journal-title":"Theranostics"},{"key":"10.1016\/j.ymthe.2026.01.030_bib17","doi-asserted-by":"crossref","first-page":"46","DOI":"10.1016\/j.freeradbiomed.2019.02.022","article-title":"Targeting dysregulation of redox homeostasis in noise-induced hearing loss: Oxidative stress and ROS signaling","volume":"135","author":"Fetoni","year":"2019","journal-title":"Free Radic. Biol. Med."},{"key":"10.1016\/j.ymthe.2026.01.030_bib18","doi-asserted-by":"crossref","first-page":"393","DOI":"10.1016\/j.jcmgh.2022.10.001","article-title":"Single-cell Transcriptomics Reveals Early Molecular and Immune Alterations Underlying the Serrated Neoplasia Pathway Toward Colorectal Cancer","volume":"15","author":"Zhou","year":"2023","journal-title":"Cell. Mol. Gastroenterol. Hepatol."},{"key":"10.1016\/j.ymthe.2026.01.030_bib19","doi-asserted-by":"crossref","first-page":"397","DOI":"10.1042\/BJ20110293","article-title":"Tetrahydrobiopterin: biochemistry and pathophysiology","volume":"438","author":"Werner","year":"2011","journal-title":"Biochem. J."},{"key":"10.1016\/j.ymthe.2026.01.030_bib20","doi-asserted-by":"crossref","first-page":"657","DOI":"10.1111\/bph.13728","article-title":"A key role for tetrahydrobiopterin-dependent endothelial NOS regulation in resistance arteries: studies in endothelial cell tetrahydrobiopterin-deficient mice","volume":"174","author":"Chuaiphichai","year":"2017","journal-title":"Br. J. Pharmacol."},{"key":"10.1016\/j.ymthe.2026.01.030_bib21","doi-asserted-by":"crossref","first-page":"585","DOI":"10.1161\/CIRCRESAHA.120.316656","article-title":"BH4 Increases nNOS Activity and Preserves Left Ventricular Function in Diabetes","volume":"128","author":"Carnicer","year":"2021","journal-title":"Circ. Res."},{"key":"10.1016\/j.ymthe.2026.01.030_bib22","doi-asserted-by":"crossref","first-page":"394","DOI":"10.1016\/j.parkreldis.2015.01.004","article-title":"Novel GCH1 variant in Dopa-responsive dystonia and Parkinson's disease","volume":"21","author":"Lewthwaite","year":"2015","journal-title":"Parkinsonism Relat. Disord."},{"key":"10.1016\/j.ymthe.2026.01.030_bib23","doi-asserted-by":"crossref","first-page":"1860","DOI":"10.1007\/s00415-018-8930-8","article-title":"GCH1 mutations in dopa-responsive dystonia and Parkinson's disease","volume":"265","author":"Yoshino","year":"2018","journal-title":"J. Neurol."},{"key":"10.1016\/j.ymthe.2026.01.030_bib24","doi-asserted-by":"crossref","first-page":"530","DOI":"10.1161\/HYPERTENSIONAHA.114.03089","article-title":"Cell-autonomous role of endothelial GTP cyclohydrolase 1 and tetrahydrobiopterin in blood pressure regulation","volume":"64","author":"Chuaiphichai","year":"2014","journal-title":"Hypertension"},{"key":"10.1016\/j.ymthe.2026.01.030_bib25","doi-asserted-by":"crossref","first-page":"1107","DOI":"10.1038\/s12276-024-01208-z","article-title":"Tetrahydrobiopterin metabolism attenuates ROS generation and radiosensitivity through LDHA S-nitrosylation: novel insight into radiogenic lung injury","volume":"56","author":"Feng","year":"2024","journal-title":"Exp. Mol. Med."},{"key":"10.1016\/j.ymthe.2026.01.030_bib26","doi-asserted-by":"crossref","first-page":"2317","DOI":"10.1016\/S0140-6736(23)02874-X","article-title":"AAV1-hOTOF gene therapy for autosomal recessive deafness 9: a single-arm trial","volume":"403","author":"Lv","year":"2024","journal-title":"Lancet"},{"key":"10.1016\/j.ymthe.2026.01.030_bib27","doi-asserted-by":"crossref","first-page":"2917","DOI":"10.1038\/s41591-025-03773-w","article-title":"AAV gene therapy for autosomal recessive deafness 9: a single-arm trial","volume":"31","author":"Qi","year":"2025","journal-title":"Nat. Med."},{"key":"10.1016\/j.ymthe.2026.01.030_bib28","doi-asserted-by":"crossref","DOI":"10.1002\/advs.202306788","article-title":"AAV-Mediated Gene Therapy Restores Hearing in Patients with DFNB9 Deafness","volume":"11","author":"Qi","year":"2024","journal-title":"Adv. Sci."},{"key":"10.1016\/j.ymthe.2026.01.030_bib29","doi-asserted-by":"crossref","first-page":"1898","DOI":"10.1038\/s41591-024-03023-5","article-title":"Bilateral gene therapy in children with autosomal recessive deafness 9: single-arm trial results","volume":"30","author":"Wang","year":"2024","journal-title":"Nat. Med."},{"key":"10.1016\/j.ymthe.2026.01.030_bib30","doi-asserted-by":"crossref","DOI":"10.1002\/advs.202306201","article-title":"Preclinical Efficacy And Safety Evaluation of AAV-OTOF in DFNB9 Mouse Model And Nonhuman Primate","volume":"11","author":"Qi","year":"2024","journal-title":"Adv. Sci."},{"key":"10.1016\/j.ymthe.2026.01.030_bib31","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1016\/S0006-8993(99)01433-X","article-title":"Expression of inducible nitric oxide synthase (iNOS\/NOS II) in the cochlea of guinea pigs after intratympanical endotoxin-treatment","volume":"830","author":"Hess","year":"1999","journal-title":"Brain Res."},{"key":"10.1016\/j.ymthe.2026.01.030_bib32","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1016\/S0378-5955(00)00018-6","article-title":"Expression of inducible nitric oxide synthase (iNOS\/NOS II) in the hydropic cochlea of guinea pigs","volume":"143","author":"Michel","year":"2000","journal-title":"Hear. Res."},{"key":"10.1016\/j.ymthe.2026.01.030_bib33","doi-asserted-by":"crossref","first-page":"106","DOI":"10.1016\/j.niox.2012.05.005","article-title":"Nitric oxide--a versatile key player in cochlear function and hearing disorders","volume":"27","author":"Heinrich","year":"2012","journal-title":"Nitric Oxide."},{"key":"10.1016\/j.ymthe.2026.01.030_bib34","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1016\/j.molcel.2019.09.018","article-title":"PTPS Facilitates Compartmentalized LTBP1 S-Nitrosylation and Promotes Tumor Growth under Hypoxia","volume":"77","author":"Zhao","year":"2020","journal-title":"Mol. Cell"},{"key":"10.1016\/j.ymthe.2026.01.030_bib35","doi-asserted-by":"crossref","first-page":"280","DOI":"10.1038\/nbt.3781","article-title":"A synthetic AAV vector enables safe and efficient gene transfer to the mammalian inner ear","volume":"35","author":"Landegger","year":"2017","journal-title":"Nat. Biotechnol."},{"key":"10.1016\/j.ymthe.2026.01.030_bib36","doi-asserted-by":"crossref","first-page":"21091","DOI":"10.1074\/jbc.273.33.21091","article-title":"GTP cyclohydrolase I inhibition by the prototypic inhibitor 2, 4-diamino-6-hydroxypyrimidine. Mechanisms and unanticipated role of GTP cyclohydrolase I feedback regulatory protein","volume":"273","author":"Xie","year":"1998","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.ymthe.2026.01.030_bib37","doi-asserted-by":"crossref","first-page":"2317","DOI":"10.1016\/S0140-6736(23)02874-X","article-title":"AAV1-hOTOF gene therapy for autosomal recessive deafness 9: a single-arm trial","volume":"403","author":"Lv","year":"2024","journal-title":"Lancet"},{"key":"10.1016\/j.ymthe.2026.01.030_bib38","doi-asserted-by":"crossref","first-page":"427","DOI":"10.1038\/s41467-018-08243-1","article-title":"AAV2.7m8 is a powerful viral vector for inner ear gene therapy","volume":"10","author":"Isgrig","year":"2019","journal-title":"Nat. Commun."},{"key":"10.1016\/j.ymthe.2026.01.030_bib39","doi-asserted-by":"crossref","DOI":"10.1016\/j.omtm.2023.101154","article-title":"Preclinical evaluation of the efficacy and safety of AAV1-hOTOF in mice and nonhuman primates","volume":"31","author":"Zhang","year":"2023","journal-title":"Mol. Ther. Methods Clin. Dev."},{"key":"10.1016\/j.ymthe.2026.01.030_bib40","doi-asserted-by":"crossref","first-page":"4341","DOI":"10.1080\/15548627.2021.1916194","article-title":"FOXG1 promotes aging inner ear hair cell survival through activation of the autophagy pathway","volume":"17","author":"He","year":"2021","journal-title":"Autophagy"},{"key":"10.1016\/j.ymthe.2026.01.030_bib41","doi-asserted-by":"crossref","DOI":"10.1016\/j.redox.2019.101406","article-title":"Noise-induced loss of sensory hair cells is mediated by ROS\/AMPKalpha pathway","volume":"29","author":"Wu","year":"2020","journal-title":"Redox Biol."},{"key":"10.1016\/j.ymthe.2026.01.030_bib42","doi-asserted-by":"crossref","DOI":"10.1016\/j.nbd.2023.106176","article-title":"P2X7 receptor is required for the ototoxicity caused by aminoglycoside in developing cochlear hair cells","volume":"183","author":"Cheng","year":"2023","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.ymthe.2026.01.030_bib43","doi-asserted-by":"crossref","first-page":"1305","DOI":"10.1016\/j.apsb.2021.11.002","article-title":"Inhibiting DNA methylation alleviates cisplatin-induced hearing loss by decreasing oxidative stress-induced mitochondria-dependent apoptosis via the LRP1-PI3K\/AKT pathway","volume":"12","author":"He","year":"2022","journal-title":"Acta Pharm. Sin. B"},{"key":"10.1016\/j.ymthe.2026.01.030_bib44","doi-asserted-by":"crossref","first-page":"1387","DOI":"10.1016\/j.ymthe.2024.02.029","article-title":"Inhibition of Gpx4-mediated ferroptosis alleviates cisplatin-induced hearing loss in C57BL\/6 mice","volume":"32","author":"Liu","year":"2024","journal-title":"Mol. Ther."},{"key":"10.1016\/j.ymthe.2026.01.030_bib45","article-title":"Lycium barbarum Glycopeptide Alleviates Neomycin-Induced Ototoxicity by Inhibiting Tryptophan Hydroxylase-Mediated Serotonin Biosynthesis","volume":"12","author":"Wu","year":"2025","journal-title":"Adv. Sci."},{"key":"10.1016\/j.ymthe.2026.01.030_bib46","doi-asserted-by":"crossref","first-page":"264","DOI":"10.3389\/fnmol.2019.00264","article-title":"Pre-treatment With Fasudil Prevents Neomycin-Induced Hair Cell Damage by Reducing the Accumulation of Reactive Oxygen Species","volume":"12","author":"Zhang","year":"2019","journal-title":"Front. Mol. Neurosci."},{"key":"10.1016\/j.ymthe.2026.01.030_bib47","article-title":"Pinoresinol Diglucoside Attenuates Nuclear Receptor Coactivator 4-Mediated Ferritinophagy Associated with Cisplatin-Induced Hearing Loss","volume":"12","author":"Chen","year":"2025","journal-title":"Adv. Sci."},{"key":"10.1016\/j.ymthe.2026.01.030_bib48","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1016\/j.sjpain.2013.12.001","article-title":"GCH1 variants, tetrahydrobiopterin and their effects on pain sensitivity","volume":"5","author":"Nasser","year":"2014","journal-title":"Scand. J. Pain"},{"key":"10.1016\/j.ymthe.2026.01.030_bib49","doi-asserted-by":"crossref","first-page":"16","DOI":"10.1186\/1471-2202-3-16","article-title":"Replicate high-density rat genome oligonucleotide microarrays reveal hundreds of regulated genes in the dorsal root ganglion after peripheral nerve injury","volume":"3","author":"Costigan","year":"2002","journal-title":"BMC Neurosci."},{"key":"10.1016\/j.ymthe.2026.01.030_bib50","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1186\/1744-8069-5-67","article-title":"Effective relief of neuropathic pain by adeno-associated virus-mediated expression of a small hairpin RNA against GTP cyclohydrolase 1","volume":"5","author":"Kim","year":"2009","journal-title":"Mol. Pain"},{"key":"10.1016\/j.ymthe.2026.01.030_bib51","doi-asserted-by":"crossref","first-page":"3400","DOI":"10.1158\/0008-5472.CAN-22-3471","article-title":"GTP Cyclohydrolase Drives Breast Cancer Development and Promotes EMT in an Enzyme-Independent Manner","volume":"83","author":"Wang","year":"2023","journal-title":"Cancer Res."},{"key":"10.1016\/j.ymthe.2026.01.030_bib52","doi-asserted-by":"crossref","DOI":"10.1126\/scitranslmed.abj1531","article-title":"Phenotypic drug screen uncovers the metabolic GCH1\/BH4 pathway as key regulator of EGFR\/KRAS-mediated neuropathic pain and lung cancer","volume":"14","author":"Cronin","year":"2022","journal-title":"Sci. Transl. Med."},{"key":"10.1016\/j.ymthe.2026.01.030_bib53","doi-asserted-by":"crossref","DOI":"10.1016\/j.phrs.2025.107781","article-title":"Inducible nitric oxide synthase (iNOS): More than an inducible enzyme? Rethinking the classification of NOS isoforms","volume":"216","author":"Farahani","year":"2025","journal-title":"Pharmacol. Res."},{"key":"10.1016\/j.ymthe.2026.01.030_bib54","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1016\/S0378-5955(02)00796-7","article-title":"Altered expression of inducible nitric oxide synthase (iNOS) in the cochlea","volume":"177","author":"Shi","year":"2003","journal-title":"Hear. Res."},{"key":"10.1016\/j.ymthe.2026.01.030_bib55","doi-asserted-by":"crossref","first-page":"124","DOI":"10.1016\/j.coviro.2017.06.003","article-title":"Host determinants of adeno-associated viral vector entry","volume":"24","author":"Pillay","year":"2017","journal-title":"Curr. Opin. Virol."},{"key":"10.1016\/j.ymthe.2026.01.030_bib56","doi-asserted-by":"crossref","first-page":"1994","DOI":"10.1056\/NEJMoa1407309","article-title":"Long-term safety and efficacy of factor IX gene therapy in hemophilia B","volume":"371","author":"Nathwani","year":"2014","journal-title":"N. Engl. J. Med."},{"key":"10.1016\/j.ymthe.2026.01.030_bib57","doi-asserted-by":"crossref","first-page":"170","DOI":"10.1016\/j.omtm.2019.08.015","article-title":"Clinical Considerations for Capsid Choice in the Development of Liver-Targeted AAV-Based Gene Transfer","volume":"15","author":"Pipe","year":"2019","journal-title":"Mol. Ther. Methods Clin. Dev."},{"key":"10.1016\/j.ymthe.2026.01.030_bib58","doi-asserted-by":"crossref","first-page":"571","DOI":"10.1080\/00016489.2025.2492366","article-title":"Cochlear inflammation in AAV-mediated gene therapy: serotype-dependent macrophage activation and promoter-driven immunogenicity","volume":"145","author":"Ni","year":"2025","journal-title":"Acta Otolaryngol."},{"key":"10.1016\/j.ymthe.2026.01.030_bib59","doi-asserted-by":"crossref","first-page":"144","DOI":"10.1016\/j.neures.2014.09.002","article-title":"Comparative analyses of adeno-associated viral vector serotypes 1, 2, 5, 8 and 9 in marmoset, mouse and macaque cerebral cortex","volume":"93","author":"Watakabe","year":"2015","journal-title":"Neurosci. Res."},{"key":"10.1016\/j.ymthe.2026.01.030_bib60","doi-asserted-by":"crossref","DOI":"10.1016\/j.omtn.2024.102135","article-title":"Hair cell-specific Myo15 promoter-mediated gene therapy rescues hearing in DFNB9 mouse model","volume":"35","author":"Wang","year":"2024","journal-title":"Mol. Ther. Nucleic Acids"},{"key":"10.1016\/j.ymthe.2026.01.030_bib61","first-page":"341","article-title":"Engineering of the AAV-Compatible Hair Cell-Specific Small-Size Myo15 Promoter for Gene Therapy in the Inner Ear","volume":"7","author":"Hu","year":"2024","journal-title":"Research (Wash D C)"},{"key":"10.1016\/j.ymthe.2026.01.030_bib62","doi-asserted-by":"crossref","first-page":"149","DOI":"10.1002\/gene.10210","article-title":"A retained selection cassette increases reporter gene expression without affecting tissue distribution in SPI3 knockout\/GFP knock-in mice","volume":"36","author":"Scarff","year":"2003","journal-title":"Genesis"},{"key":"10.1016\/j.ymthe.2026.01.030_bib63","doi-asserted-by":"crossref","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":"10.1016\/j.ymthe.2026.01.030_bib64","doi-asserted-by":"crossref","first-page":"3733","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."}],"container-title":["Molecular Therapy"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1525001626000316?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1525001626000316?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2026,6,19]],"date-time":"2026-06-19T00:33:14Z","timestamp":1781829194000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1525001626000316"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,5]]},"references-count":64,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2026,5]]}},"alternative-id":["S1525001626000316"],"URL":"https:\/\/doi.org\/10.1016\/j.ymthe.2026.01.030","relation":{},"ISSN":["1525-0016"],"issn-type":[{"value":"1525-0016","type":"print"}],"subject":[],"published":{"date-parts":[[2026,5]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Dysregulation of Serpinb6a-Gch1 axis contributes to DFNB91 deafness that is amendable to gene therapies","name":"articletitle","label":"Article Title"},{"value":"Molecular Therapy","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.ymthe.2026.01.030","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2026 The American Society of Gene and Cell Therapy. Published by Elsevier Inc. All rights are reserved, including those for text and data mining, AI training, and similar technologies.","name":"copyright","label":"Copyright"}]}}