{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,28]],"date-time":"2026-10-28T00:49:05Z","timestamp":1793148545106,"version":"4.5.0"},"reference-count":72,"publisher":"Elsevier BV","issue":"35","license":[{"start":{"date-parts":[[2009,8,1]],"date-time":"2009-08-01T00:00:00Z","timestamp":1249084800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2020,10,12]],"date-time":"2020-10-12T00:00:00Z","timestamp":1602460800000},"content-version":"vor","delay-in-days":4090,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Journal of Biological Chemistry"],"published-print":{"date-parts":[[2009,8]]},"DOI":"10.1074\/jbc.m109.012393","type":"journal-article","created":{"date-parts":[[2009,7,3]],"date-time":"2009-07-03T20:53:53Z","timestamp":1246654433000},"page":"23502-23516","source":"Crossref","is-referenced-by-count":317,"title":["Identification and Characterization of Ambroxol as an Enzyme Enhancement Agent for Gaucher Disease"],"prefix":"10.1016","volume":"284","author":[{"given":"Gustavo H.B.","family":"Maegawa","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael B.","family":"Tropak","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Justin D.","family":"Buttner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brigitte A.","family":"Rigat","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Fuller","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deepangi","family":"Pandit","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Liangiie","family":"Tang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gregory J.","family":"Kornhaber","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshitomo","family":"Hamuro","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joe T.R.","family":"Clarke","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Don J.","family":"Mahuran","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1074\/jbc.M109.012393_bib1","doi-asserted-by":"crossref","first-page":"876","DOI":"10.1136\/jmg.34.10.876-a","volume":"34","author":"Sidransky","year":"1997","journal-title":"J. Med. Genet."},{"key":"10.1074\/jbc.M109.012393_bib2","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1016\/S0065-3101(24)00049-5","volume":"44","author":"Sidransky","year":"1997","journal-title":"Adv. Pediatr."},{"key":"10.1074\/jbc.M109.012393_bib3","doi-asserted-by":"crossref","first-page":"694","DOI":"10.1007\/s00018-002-8458-y","volume":"59","author":"Zhao","year":"2002","journal-title":"Cell. Mol. Life Sci."},{"key":"10.1074\/jbc.M109.012393_bib4","doi-asserted-by":"crossref","first-page":"1353","DOI":"10.1001\/archneur.65.10.1353","volume":"65","author":"Goker-Alpan","year":"2008","journal-title":"Arch. Neurol."},{"key":"10.1074\/jbc.M109.012393_bib5","doi-asserted-by":"crossref","first-page":"937","DOI":"10.1136\/jmg.2004.024455","volume":"41","author":"Goker-Alpan","year":"2004","journal-title":"J. Med. Genet."},{"key":"10.1074\/jbc.M109.012393_bib6","doi-asserted-by":"crossref","first-page":"2277","DOI":"10.1212\/01.wnl.0000304039.11891.29","volume":"70","author":"Gan-Or","year":"2008","journal-title":"Neurology"},{"key":"10.1074\/jbc.M109.012393_bib7","doi-asserted-by":"crossref","first-page":"13813","DOI":"10.1073\/pnas.0605928103","volume":"103","author":"Steet","year":"2006","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"10.1074\/jbc.M109.012393_bib8","doi-asserted-by":"crossref","first-page":"567","DOI":"10.1002\/humu.20676","volume":"29","author":"Hruska","year":"2008","journal-title":"Hum. Mutat."},{"key":"10.1074\/jbc.M109.012393_bib9","doi-asserted-by":"crossref","first-page":"1777","DOI":"10.1086\/302925","volume":"66","author":"Koprivica","year":"2000","journal-title":"Am. J. Hum. Genet."},{"key":"10.1074\/jbc.M109.012393_bib10","doi-asserted-by":"crossref","first-page":"e37","DOI":"10.1136\/jmg.2004.028019","volume":"42","author":"Goker-Alpan","year":"2005","journal-title":"J. Med. Genet."},{"key":"10.1074\/jbc.M109.012393_bib11","doi-asserted-by":"crossref","first-page":"219","DOI":"10.1016\/j.bbadis.2004.03.007","volume":"1689","author":"Lin","year":"2004","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1074\/jbc.M109.012393_bib12","doi-asserted-by":"crossref","first-page":"519","DOI":"10.1038\/nrm2199","volume":"8","author":"Ron","year":"2007","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"10.1074\/jbc.M109.012393_bib13","doi-asserted-by":"crossref","first-page":"15428","DOI":"10.1073\/pnas.192582899","volume":"99","author":"Sawkar","year":"2002","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"10.1074\/jbc.M109.012393_bib14","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1002\/(SICI)1096-9896(199908)188:4<407::AID-PATH377>3.0.CO;2-Z","volume":"188","author":"Zimmer","year":"1999","journal-title":"J. Pathol."},{"key":"10.1074\/jbc.M109.012393_bib15","doi-asserted-by":"crossref","first-page":"4082","DOI":"10.1111\/j.1742-4658.2006.05410.x","volume":"273","author":"Chang","year":"2006","journal-title":"FEBS J."},{"key":"10.1074\/jbc.M109.012393_bib16","doi-asserted-by":"crossref","first-page":"2643","DOI":"10.1002\/cbic.200800249","volume":"9","author":"Kornhaber","year":"2008","journal-title":"ChemBioChem."},{"key":"10.1074\/jbc.M109.012393_bib17","doi-asserted-by":"crossref","first-page":"809","DOI":"10.1016\/j.cell.2007.10.025","volume":"131","author":"Wiseman","year":"2007","journal-title":"Cell"},{"key":"10.1074\/jbc.M109.012393_bib18","doi-asserted-by":"crossref","first-page":"4951","DOI":"10.1111\/j.1742-4658.2007.06040.x","volume":"274","author":"Tropak","year":"2007","journal-title":"FEBS J."},{"key":"10.1074\/jbc.M109.012393_bib19","doi-asserted-by":"crossref","first-page":"197","DOI":"10.1517\/14728214.13.1.197","volume":"13","author":"Grabowski","year":"2008","journal-title":"Expert Opin. Emerg. Drugs"},{"key":"10.1074\/jbc.M109.012393_bib20","doi-asserted-by":"crossref","first-page":"9150","DOI":"10.1074\/jbc.M609304200","volume":"282","author":"Maegawa","year":"2007","journal-title":"J. Biol. Chem."},{"key":"10.1074\/jbc.M109.012393_bib21","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1016\/j.chembiol.2006.12.006","volume":"14","author":"Tropak","year":"2007","journal-title":"Chem. Biol."},{"key":"10.1074\/jbc.M109.012393_bib22","doi-asserted-by":"crossref","first-page":"2650","DOI":"10.1002\/cbic.200800304","volume":"9","author":"Tropak","year":"2008","journal-title":"ChemBioChem."},{"key":"10.1074\/jbc.M109.012393_bib23","doi-asserted-by":"crossref","first-page":"13192","DOI":"10.1073\/pnas.0705637104","volume":"104","author":"Zheng","year":"2007","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"10.1074\/jbc.M109.012393_bib24","doi-asserted-by":"crossref","first-page":"369","DOI":"10.1016\/S0014-5793(98)01578-6","volume":"441","author":"Diettrich","year":"1998","journal-title":"FEBS Lett."},{"key":"10.1074\/jbc.M109.012393_bib25","doi-asserted-by":"crossref","first-page":"13478","DOI":"10.1074\/jbc.M308523200","volume":"279","author":"Tropak","year":"2004","journal-title":"J. Biol. Chem."},{"key":"10.1074\/jbc.M109.012393_bib26","first-page":"497","volume":"53","author":"Brown","year":"1993","journal-title":"Am. J. Hum. Genet."},{"key":"10.1074\/jbc.M109.012393_bib27","doi-asserted-by":"crossref","first-page":"26522","DOI":"10.1074\/jbc.273.41.26522","volume":"273","author":"Overkleeft","year":"1998","journal-title":"J. Biol. Chem."},{"key":"10.1074\/jbc.M109.012393_bib28","doi-asserted-by":"crossref","first-page":"212","DOI":"10.1016\/j.ymgme.2008.02.004","volume":"94","author":"Martin","year":"2008","journal-title":"Mol. Genet. Metab."},{"key":"10.1074\/jbc.M109.012393_bib29","doi-asserted-by":"crossref","first-page":"336","DOI":"10.1016\/j.ymgme.2007.08.001","volume":"92","author":"Hein","year":"2007","journal-title":"Mol. Genet. Metab."},{"key":"10.1074\/jbc.M109.012393_bib30","doi-asserted-by":"crossref","first-page":"437","DOI":"10.1016\/j.ymgme.2007.11.011","volume":"93","author":"Fuller","year":"2008","journal-title":"Mol. Genet. Metab."},{"key":"10.1074\/jbc.M109.012393_bib31","doi-asserted-by":"crossref","first-page":"497","DOI":"10.1016\/S0021-9258(18)64849-5","volume":"226","author":"Folch","year":"1957","journal-title":"J. Biol. Chem."},{"key":"10.1074\/jbc.M109.012393_bib32","series-title":"Advanced Medical Statistics","first-page":"1051","author":"Liu","year":"2003"},{"key":"10.1074\/jbc.M109.012393_bib33","first-page":"171","volume":"14","author":"Hamuro","year":"2003","journal-title":"J. Biomol. Tech."},{"key":"10.1074\/jbc.M109.012393_bib34","doi-asserted-by":"crossref","first-page":"522","DOI":"10.1002\/pro.5560020404","volume":"2","author":"Zhang","year":"1993","journal-title":"Protein Sci."},{"key":"10.1074\/jbc.M109.012393_bib35","doi-asserted-by":"crossref","first-page":"4962","DOI":"10.1021\/bi034096w","volume":"42","author":"Powell","year":"2003","journal-title":"Biochemistry"},{"key":"10.1074\/jbc.M109.012393_bib36","doi-asserted-by":"crossref","first-page":"8728","DOI":"10.1021\/ac071380a","volume":"79","author":"Tang","year":"2007","journal-title":"Anal. Chem."},{"key":"10.1074\/jbc.M109.012393_bib37","doi-asserted-by":"crossref","first-page":"1965","DOI":"10.1021\/ci800081s","volume":"48","author":"Koska","year":"2008","journal-title":"J. Chem. Inf. Model."},{"key":"10.1074\/jbc.M109.012393_bib38","first-page":"57","volume":"17","author":"Sanner","year":"1999","journal-title":"J. Mol. Graph. Model."},{"key":"10.1074\/jbc.M109.012393_bib39","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1111\/j.1432-1033.1987.tb11008.x","volume":"164","author":"Jonsson","year":"1987","journal-title":"Eur. J. Biochem."},{"key":"10.1074\/jbc.M109.012393_bib40","doi-asserted-by":"crossref","first-page":"281","DOI":"10.1006\/mgme.2000.3035","volume":"70","author":"Leonova","year":"2000","journal-title":"Mol. Genet. Metab."},{"key":"10.1074\/jbc.M109.012393_bib41","doi-asserted-by":"crossref","first-page":"219","DOI":"10.1111\/j.1365-2885.2004.00584.x","volume":"27","author":"Meijer","year":"2004","journal-title":"J. Vet. Pharmacol. Ther."},{"key":"10.1074\/jbc.M109.012393_bib42","doi-asserted-by":"crossref","first-page":"23815","DOI":"10.1074\/jbc.M502799200","volume":"280","author":"Premkumar","year":"2005","journal-title":"J. Biol. Chem."},{"key":"10.1074\/jbc.M109.012393_bib43","doi-asserted-by":"crossref","first-page":"94","DOI":"10.1021\/jm060677i","volume":"50","author":"Yu","year":"2007","journal-title":"J. Med. Chem."},{"key":"10.1074\/jbc.M109.012393_bib44","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1038\/nchembio850","volume":"3","author":"Lieberman","year":"2007","journal-title":"Nat. Chem. Biol."},{"key":"10.1074\/jbc.M109.012393_bib45","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1007\/s00439-006-0280-4","volume":"121","author":"Beck","year":"2007","journal-title":"Hum. Genet."},{"key":"10.1074\/jbc.M109.012393_bib46","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1111\/j.1651-2227.2008.00643.x","volume":"97","author":"Bembi","year":"2008","journal-title":"Acta Paediatr. Suppl."},{"key":"10.1074\/jbc.M109.012393_bib47","doi-asserted-by":"crossref","first-page":"513","DOI":"10.1023\/A:1025902113005","volume":"26","author":"Cox","year":"2003","journal-title":"J. Inherited Metab. Dis."},{"key":"10.1074\/jbc.M109.012393_bib48","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1007\/s10545-008-0779-z","volume":"31","author":"Cox","year":"2008","journal-title":"J. Inherited Metab. Dis."},{"key":"10.1074\/jbc.M109.012393_bib49","doi-asserted-by":"crossref","first-page":"4944","DOI":"10.1111\/j.1742-4658.2007.06042.x","volume":"274","author":"Yu","year":"2007","journal-title":"FEBS J."},{"key":"10.1074\/jbc.M109.012393_bib50","doi-asserted-by":"crossref","first-page":"964","DOI":"10.1038\/mt.2009.53","volume":"17","author":"Porto","year":"2009","journal-title":"Mol. Ther."},{"key":"10.1074\/jbc.M109.012393_bib51","doi-asserted-by":"crossref","first-page":"1071","DOI":"10.1016\/j.bbrc.2008.02.125","volume":"369","author":"Shen","year":"2008","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"10.1074\/jbc.M109.012393_bib52","doi-asserted-by":"crossref","first-page":"2387","DOI":"10.1093\/hmg\/ddi240","volume":"14","author":"Ron","year":"2005","journal-title":"Hum. Mol. Genet."},{"key":"10.1074\/jbc.M109.012393_bib53","doi-asserted-by":"crossref","first-page":"2310","DOI":"10.1016\/j.biocel.2005.05.008","volume":"37","author":"Schmitz","year":"2005","journal-title":"Int. J. Biochem. Cell Biol."},{"key":"10.1074\/jbc.M109.012393_bib54","doi-asserted-by":"crossref","first-page":"469","DOI":"10.1093\/hmg\/ddm324","volume":"17","author":"Wei","year":"2008","journal-title":"Hum. Mol. Genet."},{"key":"10.1074\/jbc.M109.012393_bib55","doi-asserted-by":"crossref","first-page":"229","DOI":"10.1016\/j.expneurol.2008.06.010","volume":"213","author":"Boston-Howes","year":"2008","journal-title":"Exp. Neurol."},{"key":"10.1074\/jbc.M109.012393_bib56","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1016\/j.nbd.2008.01.014","volume":"30","author":"Masuda","year":"2008","journal-title":"Neurobiol. Dis."},{"key":"10.1074\/jbc.M109.012393_bib57","doi-asserted-by":"crossref","first-page":"633","DOI":"10.1111\/j.1460-9568.2007.05663.x","volume":"26","author":"Wang","year":"2007","journal-title":"Eur. J. Neurosci."},{"key":"10.1074\/jbc.M109.012393_bib58","series-title":"Enzymes: A Practical Introduction to Structure, Mechanism, and Data Analysis","first-page":"266","author":"Copeland","year":"2000"},{"key":"10.1074\/jbc.M109.012393_bib59","doi-asserted-by":"crossref","first-page":"225","DOI":"10.1016\/j.ymgme.2008.12.008","volume":"96","author":"Rigat","year":"2009","journal-title":"Mol. Genet. Metab."},{"key":"10.1074\/jbc.M109.012393_bib60","doi-asserted-by":"crossref","first-page":"2177","DOI":"10.1016\/j.ajog.2004.07.043","volume":"191","author":"Molina","year":"2004","journal-title":"Am. J. Obstet. Gynecol."},{"key":"10.1074\/jbc.M109.012393_bib61","doi-asserted-by":"crossref","first-page":"952","DOI":"10.1183\/09031936.02.00253302","volume":"19","author":"Yang","year":"2002","journal-title":"Eur. Respir. J."},{"key":"10.1074\/jbc.M109.012393_bib62","first-page":"57S","volume":"3","author":"Wauer","year":"1989","journal-title":"Eur. Respir. J."},{"key":"10.1074\/jbc.M109.012393_bib63","doi-asserted-by":"crossref","first-page":"275","DOI":"10.1007\/BF01997619","volume":"31","author":"Bianchi","year":"1990","journal-title":"Agents Actions"},{"key":"10.1074\/jbc.M109.012393_bib64","doi-asserted-by":"crossref","first-page":"1119","DOI":"10.1517\/17425255.4.8.1119","volume":"4","author":"Malerba","year":"2008","journal-title":"Expert Opin. Drug Metab. Toxicol."},{"key":"10.1074\/jbc.M109.012393_bib65","first-page":"467","volume":"27","author":"Couet","year":"1989","journal-title":"Int. J. Clin. Pharmacol. Ther. Toxicol."},{"key":"10.1074\/jbc.M109.012393_bib66","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1016\/j.phrs.2003.07.011","volume":"49","author":"Lee","year":"2004","journal-title":"Pharmacol. Res."},{"key":"10.1074\/jbc.M109.012393_bib67","doi-asserted-by":"crossref","first-page":"237","DOI":"10.1007\/BF00271364","volume":"44","author":"Oosterhuis","year":"1993","journal-title":"Eur. J. Clin. Pharmacol."},{"key":"10.1074\/jbc.M109.012393_bib68","doi-asserted-by":"crossref","first-page":"273","DOI":"10.2165\/00044011-200323040-00007","volume":"23","author":"Rojpibulstit","year":"2003","journal-title":"Clin. Drug Invest."},{"key":"10.1074\/jbc.M109.012393_bib69","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1016\/S0378-4347(00)82313-0","volume":"513","author":"Colombo","year":"1990","journal-title":"J. Chromatogr."},{"key":"10.1074\/jbc.M109.012393_bib70","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1111\/j.1755-5949.2007.00032.x","volume":"14","author":"Weiser","year":"2008","journal-title":"CNS Neurosci. Ther."},{"key":"10.1074\/jbc.M109.012393_bib71","doi-asserted-by":"crossref","first-page":"503","DOI":"10.1203\/00006450-199205000-00019","volume":"31","author":"Tsai","year":"1992","journal-title":"Pediatr. Res."},{"key":"10.1074\/jbc.M109.012393_bib72","doi-asserted-by":"crossref","first-page":"742","DOI":"10.1373\/clinchem.2006.079012","volume":"53","author":"Groener","year":"2007","journal-title":"Clin. Chem."}],"container-title":["Journal of Biological Chemistry"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0021925818607793?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0021925818607793?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1074\/jbc.M109.012393","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,3,14]],"date-time":"2024-03-14T21:13:23Z","timestamp":1710450803000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0021925818607793"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2009,8]]},"references-count":72,"journal-issue":{"issue":"35","published-print":{"date-parts":[[2009,8]]}},"alternative-id":["S0021925818607793"],"URL":"https:\/\/doi.org\/10.1074\/jbc.m109.012393","relation":{},"ISSN":["0021-9258"],"issn-type":[{"value":"0021-9258","type":"print"}],"subject":[],"published":{"date-parts":[[2009,8]]}}}