{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,28]],"date-time":"2026-09-28T21:27:21Z","timestamp":1790630841091,"version":"4.1.0"},"reference-count":54,"publisher":"Elsevier BV","issue":"3","license":[{"start":{"date-parts":[[2024,3,1]],"date-time":"2024-03-01T00:00:00Z","timestamp":1709251200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2024,3,1]],"date-time":"2024-03-01T00:00:00Z","timestamp":1709251200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2024,2,10]],"date-time":"2024-02-10T00:00:00Z","timestamp":1707523200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100016923","name":"Hoansha Foundation","doi-asserted-by":"publisher","award":["R05B020"],"award-info":[{"award-number":["R05B020"]}],"id":[{"id":"10.13039\/100016923","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004206","name":"Osaka University","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100004206","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["JP17H05088"],"award-info":[{"award-number":["JP17H05088"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["JP22H03046"],"award-info":[{"award-number":["JP22H03046"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["jbc.org","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Journal of Biological Chemistry"],"published-print":{"date-parts":[[2024,3]]},"DOI":"10.1016\/j.jbc.2024.105743","type":"journal-article","created":{"date-parts":[[2024,2,12]],"date-time":"2024-02-12T12:09:53Z","timestamp":1707739793000},"page":"105743","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":15,"title":["Impaired lysosomal acidity maintenance in acid lipase-deficient cells leads to defective autophagy"],"prefix":"10.1016","volume":"300","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0097-8204","authenticated-orcid":false,"given":"Takahito","family":"Moriwaki","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6019-1628","authenticated-orcid":false,"given":"Seigo","family":"Terawaki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8490-7299","authenticated-orcid":false,"given":"Takanobu","family":"Otomo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.jbc.2024.105743_bib1","first-page":"1","article-title":"Lysosomal storage diseases","volume":"2","author":"Ferreira","year":"2017","journal-title":"Transl. Sci. Rare Dis."},{"key":"10.1016\/j.jbc.2024.105743_bib2","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1038\/s41572-018-0025-4","article-title":"Lysosomal storage diseases","volume":"4","author":"Platt","year":"2018","journal-title":"Nat. Rev. Dis. Primers"},{"key":"10.1016\/j.jbc.2024.105743_bib3","doi-asserted-by":"crossref","first-page":"249","DOI":"10.1001\/jama.281.3.249","article-title":"Prevalence of lysosomal storage disorders","volume":"281","author":"Meikle","year":"1999","journal-title":"JAMA"},{"key":"10.1016\/j.jbc.2024.105743_bib4","doi-asserted-by":"crossref","first-page":"1","DOI":"10.2174\/2213988501711010001","article-title":"Targeting wolman disease and cholesteryl ester storage disease: disease pathogenesis and therapeutic development","volume":"11","author":"Aguisanda","year":"2017","journal-title":"Curr. Chem. Genom. Transl. Med."},{"key":"10.1016\/j.jbc.2024.105743_bib5","series-title":"Lysosomal Acid Lipase Deficiency in GeneReviews((R))","author":"Hoffman","year":"1993"},{"key":"10.1016\/j.jbc.2024.105743_bib6","doi-asserted-by":"crossref","first-page":"218","DOI":"10.1097\/MOL.0000000000000507","article-title":"Lysosomal acid lipase and lipid metabolism: new mechanisms, new questions, and new therapies","volume":"29","author":"Zhang","year":"2018","journal-title":"Curr. Opin. Lipidol."},{"key":"10.1016\/j.jbc.2024.105743_bib7","doi-asserted-by":"crossref","first-page":"1706","DOI":"10.1074\/jbc.TM118.004165","article-title":"NPC intracellular cholesterol transporter 1 (NPC1)-mediated cholesterol export from lysosomes","volume":"294","author":"Pfeffer","year":"2019","journal-title":"J.\u00a0Biol. Chem."},{"key":"10.1016\/j.jbc.2024.105743_bib8","doi-asserted-by":"crossref","first-page":"16","DOI":"10.1186\/1750-1172-5-16","article-title":"Niemann-pick disease type C","volume":"5","author":"Vanier","year":"2010","journal-title":"Orphanet. J. Rare Dis."},{"key":"10.1016\/j.jbc.2024.105743_bib9","doi-asserted-by":"crossref","first-page":"1302","DOI":"10.1016\/j.celrep.2013.10.042","article-title":"Impaired autophagy in the lipid-storage disorder Niemann-Pick type C1 disease","volume":"5","author":"Sarkar","year":"2013","journal-title":"Cell Rep."},{"key":"10.1016\/j.jbc.2024.105743_bib10","doi-asserted-by":"crossref","first-page":"507","DOI":"10.3390\/cells11030507","article-title":"Impact of organelle transport deficits on mitophagy and autophagy in niemann-pick disease Type C","volume":"11","author":"Liedtke","year":"2022","journal-title":"Cells"},{"key":"10.1016\/j.jbc.2024.105743_bib11","doi-asserted-by":"crossref","first-page":"9442","DOI":"10.1074\/jbc.M115.636803","article-title":"beta-Cyclodextrin-threaded biocleavable polyrotaxanes ameliorate impaired autophagic flux in Niemann-Pick type C disease","volume":"290","author":"Tamura","year":"2015","journal-title":"J.\u00a0Biol. Chem."},{"key":"10.1016\/j.jbc.2024.105743_bib12","doi-asserted-by":"crossref","first-page":"5720","DOI":"10.1093\/emboj\/19.21.5720","article-title":"LC3, a mammalian homologue of yeast Apg8p, is localized in autophagosome membranes after processing","volume":"19","author":"Kabeya","year":"2000","journal-title":"EMBO J."},{"key":"10.1016\/j.jbc.2024.105743_bib13","doi-asserted-by":"crossref","first-page":"7972","DOI":"10.1073\/pnas.85.21.7972","article-title":"Bafilomycins: a class of inhibitors of membrane ATPases from microorganisms, animal cells, and plant cells","volume":"85","author":"Bowman","year":"1988","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.jbc.2024.105743_bib14","doi-asserted-by":"crossref","first-page":"110","DOI":"10.7164\/antibiotics.37.110","article-title":"Metabolic products of microorganisms. Bafilomycins, a new group of macrolide antibiotics. Production, isolation, chemical structure and biological activity","volume":"37","author":"Werner","year":"1984","journal-title":"J.\u00a0Antibiot. (Tokyo)"},{"key":"10.1016\/j.jbc.2024.105743_bib15","doi-asserted-by":"crossref","first-page":"1782","DOI":"10.1038\/s41467-021-22111-5","article-title":"Molecular basis of V-ATPase inhibition by bafilomycin A1","volume":"12","author":"Wang","year":"2021","journal-title":"Nat. Commun."},{"key":"10.1016\/j.jbc.2024.105743_bib16","doi-asserted-by":"crossref","first-page":"2","DOI":"10.1128\/MCB.06159-11","article-title":"Role of AMPK-mTOR-Ulk1\/2 in the regulation of autophagy: cross talk, shortcuts, and feedbacks","volume":"32","author":"Alers","year":"2012","journal-title":"Mol. Cell Biol."},{"key":"10.1016\/j.jbc.2024.105743_bib17","doi-asserted-by":"crossref","DOI":"10.7554\/eLife.78923","article-title":"A\u00a0pulse-chasable reporter processing assay for mammalian autophagic flux with HaloTag","volume":"11","author":"Yim","year":"2022","journal-title":"Elife"},{"key":"10.1016\/j.jbc.2024.105743_bib18","doi-asserted-by":"crossref","first-page":"207","DOI":"10.1080\/15548627.2017.1378838","article-title":"Autophagy pathway: cellular and molecular mechanisms","volume":"14","author":"Yu","year":"2018","journal-title":"Autophagy"},{"key":"10.1016\/j.jbc.2024.105743_bib19","doi-asserted-by":"crossref","first-page":"452","DOI":"10.4161\/auto.4451","article-title":"Dissection of the autophagosome maturation process by a novel reporter protein, tandem fluorescent-tagged LC3","volume":"3","author":"Kimura","year":"2007","journal-title":"Autophagy"},{"key":"10.1016\/j.jbc.2024.105743_bib20","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1007\/s10616-012-9468-8","article-title":"Advantages of AlaGln as an additive to cell culture medium: use with anti-CD20 chimeric antibody-producing POTELLIGENT CHO cell lines","volume":"65","author":"Imamoto","year":"2013","journal-title":"Cytotechnology"},{"key":"10.1016\/j.jbc.2024.105743_bib21","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1016\/S0928-0987(98)00012-8","article-title":"Degradation kinetics of L-alanyl-L-glutamine and its derivatives in aqueous solution","volume":"7","author":"Arii","year":"1999","journal-title":"Eur. J. Pharm. Sci."},{"key":"10.1016\/j.jbc.2024.105743_bib22","doi-asserted-by":"crossref","first-page":"375","DOI":"10.1007\/s00217-010-1383-7","article-title":"Pyroglutamic acid development during grape must cooking","volume":"232","author":"Montevecchi","year":"2010","journal-title":"Eur. Food Res. Technol."},{"key":"10.1016\/j.jbc.2024.105743_bib23","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1038\/s41580-018-0003-4","article-title":"Mechanism and medical implications of mammalian autophagy","volume":"19","author":"Dikic","year":"2018","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"10.1016\/j.jbc.2024.105743_bib24","doi-asserted-by":"crossref","first-page":"1097","DOI":"10.1002\/jimd.12061","article-title":"Ammonia and autophagy: an emerging relationship with implications for disorders with hyperammonemia","volume":"42","author":"Soria","year":"2019","journal-title":"J.\u00a0Inherit. Metab. Dis."},{"key":"10.1016\/j.jbc.2024.105743_bib25","doi-asserted-by":"crossref","DOI":"10.1042\/BSR20200905","article-title":"Chloroquine and bafilomycin A mimic lysosomal storage disorders and impair mTORC1 signalling","volume":"40","author":"Fedele","year":"2020","journal-title":"Biosci. Rep."},{"key":"10.1016\/j.jbc.2024.105743_bib26","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1016\/j.arr.2016.05.004","article-title":"Disorders of lysosomal acidification-The emerging role of v-ATPase in aging and neurodegenerative disease","volume":"32","author":"Colacurcio","year":"2016","journal-title":"Ageing Res. Rev."},{"key":"10.1016\/j.jbc.2024.105743_bib27","doi-asserted-by":"crossref","DOI":"10.3390\/cells10123537","article-title":"Ion channels and pumps in autophagy: a reciprocal relationship","volume":"10","author":"Abuammar","year":"2021","journal-title":"Cells"},{"key":"10.1016\/j.jbc.2024.105743_bib28","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1186\/s12943-018-0811-3","article-title":"The curious case of vacuolar ATPase: regulation of signaling pathways","volume":"17","author":"Pamarthy","year":"2018","journal-title":"Mol. Cancer"},{"key":"10.1016\/j.jbc.2024.105743_bib29","article-title":"Ammonia induces autophagy through dopamine receptor D3 and MTOR","volume":"11","author":"Li","year":"2016","journal-title":"PLoS One"},{"key":"10.1016\/j.jbc.2024.105743_bib30","doi-asserted-by":"crossref","first-page":"247","DOI":"10.1016\/j.ab.2004.01.010","article-title":"Quantitation of the lysosomotropic character of cationic amphiphilic drugs using the fluorescent basic amine Red DND-99","volume":"327","author":"Lemieux","year":"2004","journal-title":"Anal. Biochem."},{"key":"10.1016\/j.jbc.2024.105743_bib31","doi-asserted-by":"crossref","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":"10.1016\/j.jbc.2024.105743_bib32","doi-asserted-by":"crossref","DOI":"10.3389\/fcell.2021.790568","article-title":"Alterations in lysosome homeostasis in lipid-related disorders: impact on metabolic tissues and immune cells","volume":"9","author":"Cabrera-Reyes","year":"2021","journal-title":"Front. Cell Dev. Biol."},{"key":"10.1016\/j.jbc.2024.105743_bib33","doi-asserted-by":"crossref","first-page":"119","DOI":"10.1093\/hmg\/ddm289","article-title":"A\u00a0block of autophagy in lysosomal storage disorders","volume":"17","author":"Settembre","year":"2008","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.jbc.2024.105743_bib34","doi-asserted-by":"crossref","first-page":"1209","DOI":"10.1083\/jcb.200811005","article-title":"Cholesterol sensor ORP1L contacts the ER protein VAP to control Rab7-RILP-p150 Glued and late endosome positioning","volume":"185","author":"Rocha","year":"2009","journal-title":"J.\u00a0Cell Biol."},{"key":"10.1016\/j.jbc.2024.105743_bib35","first-page":"842","article-title":"The lysosomal storage disorders mucolipidosis type II, type III alpha\/beta, and type III gamma: update on GNPTAB and GNPTG mutations","volume":"40","author":"Velho","year":"2019","journal-title":"Hum. Mutat."},{"key":"10.1016\/j.jbc.2024.105743_bib36","doi-asserted-by":"crossref","first-page":"35283","DOI":"10.1074\/jbc.M111.267930","article-title":"Lysosomal storage causes cellular dysfunction in mucolipidosis II skin fibroblasts","volume":"286","author":"Otomo","year":"2011","journal-title":"J.\u00a0Biol. Chem."},{"key":"10.1016\/j.jbc.2024.105743_bib37","series-title":"Niemann-Pick Disease Type C in GeneReviews((R))","author":"Patterson","year":"1993"},{"key":"10.1016\/j.jbc.2024.105743_bib38","doi-asserted-by":"crossref","first-page":"665","DOI":"10.1083\/jcb.90.3.665","article-title":"Effect of weak bases on the intralysosomal pH in mouse peritoneal macrophages","volume":"90","author":"Poole","year":"1981","journal-title":"J.\u00a0Cell Biol."},{"key":"10.1016\/j.jbc.2024.105743_bib39","doi-asserted-by":"crossref","first-page":"777","DOI":"10.1007\/s11427-016-5090-x","article-title":"Regulation of lysosomal ion homeostasis by channels and transporters","volume":"59","author":"Xiong","year":"2016","journal-title":"Sci. China Life Sci."},{"key":"10.1016\/j.jbc.2024.105743_bib40","doi-asserted-by":"crossref","first-page":"1444","DOI":"10.2215\/CJN.10311013","article-title":"Urea and ammonia metabolism and the control of renal nitrogen excretion","volume":"10","author":"Weiner","year":"2015","journal-title":"Clin. J. Am. Soc. Nephrol."},{"key":"10.1016\/j.jbc.2024.105743_bib41","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1111\/j.1939-1676.1994.tb03201.x","article-title":"Ammonia metabolism and the urea cycle: function and clinical implications","volume":"8","author":"Dimski","year":"1994","journal-title":"J.\u00a0Vet. Intern. Med."},{"key":"10.1016\/j.jbc.2024.105743_bib42","doi-asserted-by":"crossref","first-page":"369","DOI":"10.1265\/jjh.19.369","article-title":"Metabolism of ammonia under various environmental conditions 1. Chages in the ammonia content of brain, liver, and blood under some environmental conditions","volume":"19","author":"Sakaguchi","year":"1965","journal-title":"Nihon Eiseigaku Zasshi"},{"key":"10.1016\/j.jbc.2024.105743_bib43","doi-asserted-by":"crossref","first-page":"1705","DOI":"10.1111\/j.1537-2995.2006.00962.x","article-title":"Acquired lysosomal storage caused by frequent plasmapheresis procedures with hydroxyethyl starch","volume":"46","author":"Auwerda","year":"2006","journal-title":"Transfusion"},{"key":"10.1016\/j.jbc.2024.105743_bib44","doi-asserted-by":"crossref","first-page":"602","DOI":"10.1016\/j.bbalip.2012.08.013","article-title":"Drug induced phospholipidosis: an acquired lysosomal storage disorder","volume":"1831","author":"Shayman","year":"2013","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.jbc.2024.105743_bib45","doi-asserted-by":"crossref","first-page":"1534","DOI":"10.1681\/ASN.2016070731","article-title":"High-fat diet-induced lysosomal dysfunction and impaired autophagic flux contribute to lipotoxicity in the kidney","volume":"28","author":"Yamamoto","year":"2017","journal-title":"J.\u00a0Am. Soc. Nephrol."},{"key":"10.1016\/j.jbc.2024.105743_bib46","doi-asserted-by":"crossref","first-page":"470","DOI":"10.1002\/path.5150","article-title":"Excessive dietary lipid intake provokes an acquired form of lysosomal lipid storage disease in the kidney","volume":"246","author":"Rampanelli","year":"2018","journal-title":"J.\u00a0Pathol."},{"key":"10.1016\/j.jbc.2024.105743_bib47","doi-asserted-by":"crossref","first-page":"186","DOI":"10.1016\/0261-5614(91)90037-D","article-title":"Factors affecting the stability of L-glutamine in solution","volume":"10","author":"Khan","year":"1991","journal-title":"Clin. Nutr."},{"key":"10.1016\/j.jbc.2024.105743_bib48","doi-asserted-by":"crossref","first-page":"1171","DOI":"10.1007\/s10616-015-9875-8","article-title":"Stability of minimum essential medium functionality despite L-glutamine decomposition","volume":"68","author":"Jagusic","year":"2016","journal-title":"Cytotechnology"},{"key":"10.1016\/j.jbc.2024.105743_bib49","doi-asserted-by":"crossref","first-page":"623","DOI":"10.1038\/nrm2745","article-title":"Lysosome biogenesis and lysosomal membrane proteins: trafficking meets function","volume":"10","author":"Saftig","year":"2009","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"10.1016\/j.jbc.2024.105743_bib50","doi-asserted-by":"crossref","first-page":"4329","DOI":"10.1242\/jcs.196287","article-title":"Mechanisms and functions of lysosome positioning","volume":"129","author":"Pu","year":"2016","journal-title":"J.\u00a0Cell Sci."},{"key":"10.1016\/j.jbc.2024.105743_bib51","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1080\/15548627.2020.1797280","article-title":"Guidelines for the use and interpretation of assays for monitoring autophagy (4th edition)","volume":"17","author":"Klionsky","year":"2021","journal-title":"Autophagy"},{"key":"10.1016\/j.jbc.2024.105743_bib52","doi-asserted-by":"crossref","first-page":"1390","DOI":"10.1111\/tra.12227","article-title":"LYST affects lysosome size and quantity, but not trafficking or degradation through autophagy or endocytosis","volume":"15","author":"Holland","year":"2014","journal-title":"Traffic"},{"key":"10.1016\/j.jbc.2024.105743_bib53","doi-asserted-by":"crossref","first-page":"71","DOI":"10.3389\/fcell.2017.00071","article-title":"Live-cell microscopy and fluorescence-based measurement of luminal pH in intracellular organelles","volume":"5","author":"Ma","year":"2017","journal-title":"Front. Cell Dev. Biol."},{"key":"10.1016\/j.jbc.2024.105743_bib54","doi-asserted-by":"crossref","first-page":"359","DOI":"10.1016\/j.ab.2008.10.007","article-title":"Using LysoSensor Yellow\/Blue DND-160 to sense acidic pH under high hydrostatic pressures","volume":"384","author":"DePedro","year":"2009","journal-title":"Anal. Biochem."}],"container-title":["Journal of Biological Chemistry"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0021925824001194?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0021925824001194?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2026,4,28]],"date-time":"2026-04-28T21:44:56Z","timestamp":1777412696000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0021925824001194"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,3]]},"references-count":54,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2024,3]]}},"alternative-id":["S0021925824001194"],"URL":"https:\/\/doi.org\/10.1016\/j.jbc.2024.105743","relation":{},"ISSN":["0021-9258"],"issn-type":[{"value":"0021-9258","type":"print"}],"subject":[],"published":{"date-parts":[[2024,3]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Impaired lysosomal acidity maintenance in acid lipase-deficient cells leads to defective autophagy","name":"articletitle","label":"Article Title"},{"value":"Journal of Biological Chemistry","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.jbc.2024.105743","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2024 The Authors. Published by Elsevier Inc on behalf of American Society for Biochemistry and Molecular Biology.","name":"copyright","label":"Copyright"}],"article-number":"105743"}}