{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,22]],"date-time":"2026-06-22T03:50:08Z","timestamp":1782100208978,"version":"3.54.5"},"reference-count":26,"publisher":"Springer Science and Business Media LLC","issue":"7","license":[{"start":{"date-parts":[[2026,4,17]],"date-time":"2026-04-17T00:00:00Z","timestamp":1776384000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.springernature.com\/gp\/researchers\/text-and-data-mining"},{"start":{"date-parts":[[2026,4,17]],"date-time":"2026-04-17T00:00:00Z","timestamp":1776384000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.springernature.com\/gp\/researchers\/text-and-data-mining"}],"funder":[{"name":"Guizhou Provincial Science and Technology Program Project","award":["No. ZK[2024]667"],"award-info":[{"award-number":["No. ZK[2024]667"]}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Appl Biochem Biotechnol"],"published-print":{"date-parts":[[2026,7]]},"DOI":"10.1007\/s12010-025-05579-5","type":"journal-article","created":{"date-parts":[[2026,4,17]],"date-time":"2026-04-17T03:03:40Z","timestamp":1776395020000},"page":"5330-5345","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["ATP6V1B2 Regulating Autophagy Flux To Affect Abnormal Auditory Development"],"prefix":"10.1007","volume":"198","author":[{"given":"ZiFang","family":"Feng","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jie","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"YunZhong","family":"Yang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"MingLu","family":"Chen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"XueMei","family":"Wu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0004-4437-4693","authenticated-orcid":false,"given":"ChengYing","family":"Zhou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,4,17]]},"reference":[{"issue":"3","key":"5579_CR1","doi-asserted-by":"publisher","first-page":"317","DOI":"10.1684\/epd.2020.1166","volume":"22","author":"LM Inuzuka","year":"2020","unstructured":"Inuzuka, L. M., Macedo-Souza, L. I., Della-Rippa, B., Monteiro, F. P., Delgado, D. S., Godoy, L. F., Ramos, L., de Athayde Costa, L. S., Garzon, E., & Kok, F. (2020). ATP6V1B2-related epileptic encephalopathy. Epileptic Disorders : International Epilepsy Journal with Videotape, 22(3), 317\u2013322.","journal-title":"Epileptic Disorders : International Epilepsy Journal with Videotape"},{"issue":"10","key":"5579_CR2","doi-asserted-by":"publisher","first-page":"588","DOI":"10.1016\/j.braindev.2023.07.004","volume":"45","author":"G Amore","year":"2023","unstructured":"Amore, G., Cal\u00ec, E., Span\u00f2, M., Ceravolo, G., Mangano, G. D., Scorrano, G., Efthymiou, S., Salpietro, V., Houlden, H., & Di Rosa, G. (2023). ATP6V1B2-related disorders featuring Lennox-Gastaut-syndrome: A case-based overview. Brain Development, 45(10), 588\u2013596.","journal-title":"Brain Development"},{"key":"5579_CR3","doi-asserted-by":"publisher","first-page":"742714","DOI":"10.3389\/fcell.2021.742714","volume":"9","author":"S Qiu","year":"2021","unstructured":"Qiu, S., Zhao, W., Gao, X., Li, D., Wang, W., Gao, B., Han, W., Yang, S., Dai, P., Cao, P., & Yuan, Y. (2021). Syndromic deafness gene ATP6V1B2 controls degeneration of spiral ganglion neurons through modulating proton flux. Front Cell Dev Biol, 9, 742714.","journal-title":"Front Cell Dev Biol"},{"issue":"6","key":"5579_CR4","doi-asserted-by":"publisher","first-page":"1192","DOI":"10.1080\/15548627.2024.2448913","volume":"21","author":"C Song","year":"2025","unstructured":"Song, C., Dong, Q., Yao, Y., Cui, Y., Zhang, C., Lin, L., Zhu, L., Hu, Y., Liu, H., Jin, Y., Li, P., Liu, X., & Cao, C. (2025). Nonreceptor tyrosine kinase ABL1 regulates lysosomal acidification by phosphorylating the ATP6V1B2 subunit of the vacuolar-type H+-ATPase. Autophagy, 21(6), 1192\u20131211.","journal-title":"Autophagy"},{"key":"5579_CR5","doi-asserted-by":"publisher","first-page":"176759","DOI":"10.1016\/j.ejphar.2024.176759","volume":"978","author":"GJ Zou","year":"2024","unstructured":"Zou, G. J., Chen, Z. R., Wang, X. Q., et al. (2024). Microglial activation in the medial prefrontal cortex after remote fear recall participates in the regulation of auditory fear extinction. European Journal of Pharmacology, 978, 176759.","journal-title":"European Journal of Pharmacology"},{"key":"5579_CR6","doi-asserted-by":"publisher","unstructured":"Beauregard-Lacroix, E., Pacheco-Cuellar, G., Ajeawung, N. F., Tardif, J., Dieterich, K., Dabir, T., Vind-Kezunovic, D., White, S. M., Zadori, D., Castiglioni, C., Tranebj\u00e6rg, L., T\u00f8rring, P. M., Blair, E., Wisniewska, M., Camurri, M. V., van Bever, Y., Molidperee, S., Taylor, J., Dionne-Laporte, A., Sisodiya, S. M., Hennekam, R. C. M., & Campeau, P. M. (2021). DOORS syndrome and a recurrent truncating ATP6V1B2 variant. Genet Med. ;23(1):149\u2013154. https:\/\/doi.org\/10.1038\/s41436-020-00950-9. Epub 2020 Sep 2. Erratum in: Genet Med. 2021;23(1):237.","DOI":"10.1038\/s41436-020-00950-9"},{"issue":"3","key":"5579_CR7","doi-asserted-by":"publisher","first-page":"327","DOI":"10.1002\/ajmg.c.31412","volume":"166C","author":"PM Campeau","year":"2014","unstructured":"Campeau, P. M., Hennekam, R. C., & DOORS syndrome collaborative group. (2014). DOORS syndrome: phenotype, genotype and comparison with Coffin-Siris syndrome. Am J Med Genet C Semin Med Genet, 166C(3), 327\u2013332.","journal-title":"Am J Med Genet C Semin Med Genet"},{"issue":"3","key":"5579_CR8","doi-asserted-by":"publisher","first-page":"496","DOI":"10.1080\/15548627.2021.1943177","volume":"18","author":"C Deneubourg","year":"2022","unstructured":"Deneubourg, C., Ramm, M., Smith, L. J., Baron, O., Singh, K., Byrne, S. C., Duchen, M. R., Gautel, M., Eskelinen, E. L., Fanto, M., & Jungbluth, H. (2022). The spectrum of neurodevelopmental, neuromuscular and neurodegenerative disorders due to defective autophagy. Autophagy, 18(3), 496\u2013517.","journal-title":"Autophagy"},{"issue":"1","key":"5579_CR9","doi-asserted-by":"publisher","first-page":"470","DOI":"10.1007\/s11033-025-10577-1","volume":"52","author":"X Zhang","year":"2025","unstructured":"Zhang, X., Wu, Z., Zhou, X., & Tao, H. (2025). Mitochondrial dysfunction in epilepsy: Mechanistic insights and clinical strategies. Molecular Biology Reports, 52(1), 470.","journal-title":"Molecular Biology Reports"},{"issue":"1","key":"5579_CR10","doi-asserted-by":"publisher","first-page":"15","DOI":"10.1080\/15548627.2023.2250217","volume":"20","author":"EI Lewerissa","year":"2024","unstructured":"Lewerissa, E. I., Nadif Kasri, N., & Linda, K. (2024). Epigenetic regulation of autophagy-related genes: Implications for neurodevelopmental disorders. Autophagy, 20(1), 15\u201328.","journal-title":"Autophagy"},{"issue":"1","key":"5579_CR11","doi-asserted-by":"publisher","first-page":"142","DOI":"10.1186\/s10020-023-00742-2","volume":"29","author":"NH Ali","year":"2023","unstructured":"Ali, N. H., Al-Kuraishy, H. M., Al-Gareeb, A. I., et al. (2023). Autophagy and autophagy signaling in epilepsy: Possible role of autophagy activator. Molecular Medicine, 29(1), 142. Published 2023 Oct 25.","journal-title":"Molecular Medicine"},{"issue":"4","key":"5579_CR12","first-page":"100349","volume":"5","author":"G Carpentieri","year":"2024","unstructured":"Carpentieri, G., Cecchetti, S., Bocchinfuso, G., Radio, F. C., Leoni, C., Onesimo, R., Calligari, P., Pietrantoni, A., Ciolfi, A., Ferilli, M., Calderan, C., Cappuccio, G., Martinelli, S., Messina, E., Caputo, V., H\u00fcffmeier, U., Mignot, C., Auvin, S., Capri, Y., Lourenco, C. M., Russell, B. E., Neustad, A., Brunetti Pierri, N., Keren, B., Reis, A., Cohen, J. S., Heidlebaugh, A., Smith, C., Thiel, C. T., Salviati, L., Zampino, G., Campeau, P. M., Stella, L., Tartaglia, M., & Flex, E. (2024). Dominantly acting variants in ATP6V1C1 and ATP6V1B2 cause a multisystem phenotypic spectrum by altering lysosomal and\/or autophagosome function. HGG Adv, 5(4), 100349.","journal-title":"HGG Adv"},{"issue":"3","key":"5579_CR13","doi-asserted-by":"publisher","first-page":"975","DOI":"10.1159\/000488827","volume":"46","author":"R Lei","year":"2018","unstructured":"Lei, R., Zhao, F., Tang, C. Y., Luo, M., Yang, S. K., Cheng, W., Li, X. W., & Duan, S. B. (2018). Mitophagy plays a protective role in iodinated Contrast-Induced acute renal tubular epithelial cells injury. Cellular Physiology and Biochemistry, 46(3), 975\u2013985.","journal-title":"Cellular Physiology and Biochemistry"},{"issue":"6","key":"5579_CR14","doi-asserted-by":"publisher","first-page":"BSR20180876","DOI":"10.1042\/BSR20180876","volume":"38","author":"X Yang","year":"2018","unstructured":"Yang, X., Yan, X., Yang, D., Zhou, J., Song, J., & Yang, D. (2018). Rapamycin attenuates mitochondrial injury and renal tubular cell apoptosis in experimental contrast-induced acute kidney injury in rats. Bioscience Reports, 38(6), BSR20180876.","journal-title":"Bioscience Reports"},{"issue":"7","key":"5579_CR15","doi-asserted-by":"publisher","first-page":"1187","DOI":"10.1016\/j.cell.2017.05.045","volume":"169","author":"IA Roundtree","year":"2017","unstructured":"Roundtree, I. A., Evans, M. E., Pan, T., & He, C. (2017). Dynamic RNA modifications in gene expression regulation. Cell, 169(7), 1187\u20131200.","journal-title":"Cell"},{"key":"5579_CR16","doi-asserted-by":"crossref","unstructured":"Lystad, A. H. (2025). V-ATPase assembly at leaky lysosomes. Nature Structural & Molecular Biology, 32(10), 1862\u20134.","DOI":"10.1038\/s41594-025-01612-7"},{"issue":"1","key":"5579_CR17","first-page":"131","volume":"20","author":"Y Sato","year":"1993","unstructured":"Sato, Y., Ohtsuka, K., & Ono, K. (1993). [Concentration of mitomycin C in portal blood and peripheral blood after intra venous, intraperitoneal or intrapelvic administration]. Gan To Kagaku Ryoho, 20(1), 131\u2013136.","journal-title":"Gan To Kagaku Ryoho"},{"issue":"9","key":"5579_CR18","doi-asserted-by":"publisher","first-page":"1281","DOI":"10.3109\/0886022X.2013.824362","volume":"35","author":"A Subasioglu Uzak","year":"2013","unstructured":"Subasioglu Uzak, A., Cakar, N., Comak, E., Yalcinkaya, F., & Tekin, M. (2013). ATP6V1B1 mutations in distal renal tubular acidosis and sensorineural hearing loss: Clinical and genetic spectrum of five families. Renal Failure, 35(9), 1281\u20131284.","journal-title":"Renal Failure"},{"issue":"3","key":"5579_CR19","doi-asserted-by":"publisher","first-page":"274","DOI":"10.1111\/j.1399-0004.2012.01891.x","volume":"83","author":"N Mohebbi","year":"2013","unstructured":"Mohebbi, N., Vargas-Poussou, R., Hegemann, S. C., Schuknecht, B., Kistler, A. D., W\u00fcthrich, R. P., & Wagner, C. A. (2013). Homozygous and compound heterozygous mutations in the ATP6V1B1 gene in patients with renal tubular acidosis and sensorineural hearing loss. Clinical Genetics, 83(3), 274\u2013278.","journal-title":"Clinical Genetics"},{"issue":"8","key":"5579_CR20","first-page":"e17349","volume":"13","author":"MV Batista","year":"2021","unstructured":"Batista, M. V., Ulrich, J., Costa, L., & Ribeiro, L. A. (2021). Multiple primary malignancies in head and neck cancer: A university hospital experience over a Five-Year period. Cureus, 13(8), e17349.","journal-title":"Cureus"},{"key":"5579_CR21","doi-asserted-by":"publisher","first-page":"251","DOI":"10.1016\/j.freeradbiomed.2024.11.035","volume":"226","author":"B Chen","year":"2025","unstructured":"Chen, B., Liu, Y., Luo, S., Zhou, J., Wang, Y., He, Q., Zhuang, G., Hao, H., Ma, F., Xiao, X., & Li, S. (2025). Itaconic acid ameliorates necrotizing Enterocolitis through the TFEB-mediated autophagy-lysosomal pathway. Free Radical Biology and Medicine, 226, 251\u2013265.","journal-title":"Free Radical Biology and Medicine"},{"issue":"3","key":"5579_CR22","doi-asserted-by":"publisher","first-page":"e70066","DOI":"10.1111\/aji.70066","volume":"93","author":"L Xiao","year":"2025","unstructured":"Xiao, L., Mei, Z., Chen, J., Zhao, K., Zhang, H., Sharma, S., Liao, A., & Liu, C. (2025). Targeted degradation technology based on the Autophagy-Lysosomal pathway: A promising strategy for treating preeclampsia. American Journal of Reproductive Immunology, 93(3), e70066.","journal-title":"American Journal of Reproductive Immunology"},{"issue":"3","key":"5579_CR23","doi-asserted-by":"publisher","first-page":"839","DOI":"10.1080\/15548627.2022.2105562","volume":"19","author":"W Zheng","year":"2023","unstructured":"Zheng, W., Chen, Q., Liu, H., Zeng, L., Zhou, Y., Liu, X., Bai, Y., Zhang, J., Pan, Y., & Shao, C. (2023). SDC1-dependent TGM2 determines radiosensitivity in glioblastoma by coordinating EPG5-mediated fusion of autophagosomes with lysosomes. Autophagy, 19(3), 839\u2013857.","journal-title":"Autophagy"},{"key":"5579_CR24","doi-asserted-by":"publisher","first-page":"skac393","DOI":"10.1093\/jas\/skac393","volume":"101","author":"K Ma","year":"2023","unstructured":"Ma, K., Chen, N., Wang, H., Li, Q., Shi, H., Su, M., Zhang, Y., Ma, Y., & Li, T. (2023). The regulatory role of BMP4 in testicular Sertoli cells of Tibetan sheep. Journal of Animal Science, 101, skac393.","journal-title":"Journal of Animal Science"},{"issue":"1","key":"5579_CR25","doi-asserted-by":"publisher","first-page":"673","DOI":"10.1007\/s12672-024-01578-w","volume":"15","author":"Y Pan","year":"2024","unstructured":"Pan, Y., Chen, H., Lv, C., He, W., Xu, Y., & Xuan, Q. (2024). ATP6V1C1, associated with the tumor microenvironment and mTORC1 signaling pathway, is a potential diagnostic, prognostic, and therapeutic biomarker for hepatocellular carcinoma. Discov Oncol, 15(1), 673.","journal-title":"Discov Oncol"},{"issue":"1","key":"5579_CR26","doi-asserted-by":"publisher","first-page":"e84833","DOI":"10.1371\/journal.pone.0084833","volume":"9","author":"M Cai","year":"2014","unstructured":"Cai, M., Liu, P., Wei, L., Wang, J., Qi, J., Feng, S., & Deng, L. (2014). Atp6v1c1 May regulate filament actin arrangement in breast cancer cells. PLoS One, 9(1), e84833. https:\/\/doi.org\/10.1371\/journal.pone.0084833. Erratum in: PLoS One. 2014;9(1):e84833.","journal-title":"PLoS One"}],"container-title":["Applied Biochemistry and Biotechnology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s12010-025-05579-5.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s12010-025-05579-5","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s12010-025-05579-5.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,6,22]],"date-time":"2026-06-22T03:29:02Z","timestamp":1782098942000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s12010-025-05579-5"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,4,17]]},"references-count":26,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2026,7]]}},"alternative-id":["5579"],"URL":"https:\/\/doi.org\/10.1007\/s12010-025-05579-5","relation":{},"ISSN":["0273-2289","1559-0291"],"issn-type":[{"value":"0273-2289","type":"print"},{"value":"1559-0291","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,4,17]]},"assertion":[{"value":"30 December 2025","order":1,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"17 April 2026","order":2,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"All animal experiments were complied with the ARRIVE guidelines and performed in accordance with the National Institutes of Health Guide for the Care and Use of Laboratory Animals. The experiments were approved by the Animal Experimental Ethical Inspection Form of Guizhou Medical University.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical Statement"}},{"value":"The authors have no conflicts of interest to declare.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}]}}