{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,3]],"date-time":"2026-10-03T10:41:19Z","timestamp":1791024079766,"version":"4.1.0"},"reference-count":40,"publisher":"Elsevier BV","issue":"14","license":[{"start":{"date-parts":[[2004,4,1]],"date-time":"2004-04-01T00:00:00Z","timestamp":1080777600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2020,9,30]],"date-time":"2020-09-30T00:00:00Z","timestamp":1601424000000},"content-version":"vor","delay-in-days":6026,"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":[[2004,4]]},"DOI":"10.1074\/jbc.m308523200","type":"journal-article","created":{"date-parts":[[2004,3,26]],"date-time":"2004-03-26T19:48:39Z","timestamp":1080330519000},"page":"13478-13487","source":"Crossref","is-referenced-by-count":180,"title":["Pharmacological Enhancement of \u03b2-Hexosaminidase Activity in Fibroblasts from Adult Tay-Sachs and Sandhoff Patients"],"prefix":"10.1016","volume":"279","author":[{"given":"Michael B.","family":"Tropak","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen P.","family":"Reid","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marianne","family":"Guiral","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen G.","family":"Withers","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Don","family":"Mahuran","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1074\/jbc.M308523200_bib1","series-title":"The Metabolic and Molecular Bases of Inherited Disease","first-page":"2839","volume":"2","author":"Gravel","year":"1995"},{"key":"10.1074\/jbc.M308523200_bib2","doi-asserted-by":"crossref","first-page":"1093","DOI":"10.1016\/S0022-2836(03)00216-X","volume":"327","author":"Mark","year":"2003","journal-title":"J. Mol. Biol."},{"key":"10.1074\/jbc.M308523200_bib3","doi-asserted-by":"crossref","first-page":"3963","DOI":"10.1021\/bi9524575","volume":"35","author":"Hou","year":"1996","journal-title":"Biochemistry"},{"key":"10.1074\/jbc.M308523200_bib4","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1016\/0925-4439(91)90044-A","volume":"1096","author":"Mahuran","year":"1991","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1074\/jbc.M308523200_bib5","doi-asserted-by":"crossref","first-page":"17921","DOI":"10.1016\/S0021-9258(18)38251-6","volume":"265","author":"Dlott","year":"1990","journal-title":"J. Biol. Chem."},{"key":"10.1074\/jbc.M308523200_bib6","doi-asserted-by":"crossref","first-page":"14975","DOI":"10.1074\/jbc.272.23.14975","volume":"272","author":"Cao","year":"1997","journal-title":"J. Biol. Chem."},{"key":"10.1074\/jbc.M308523200_bib7","doi-asserted-by":"crossref","first-page":"58","DOI":"10.1159\/000112332","volume":"6","author":"Conzelmann","year":"1984","journal-title":"Dev. Neurosci."},{"key":"10.1074\/jbc.M308523200_bib8","doi-asserted-by":"crossref","first-page":"355","DOI":"10.1016\/S0165-6147(03)00158-5","volume":"24","author":"Fan","year":"2003","journal-title":"Trends Pharmacol. Sci."},{"key":"10.1074\/jbc.M308523200_bib9","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1038\/nrm1052","volume":"4","author":"Ellgaard","year":"2003","journal-title":"Nat. Rev. Mol. Cell. Biol."},{"key":"10.1074\/jbc.M308523200_bib10","first-page":"497","volume":"53","author":"Brown","year":"1993","journal-title":"Am. J. Hum. Genet."},{"key":"10.1074\/jbc.M308523200_bib11","doi-asserted-by":"crossref","first-page":"371","DOI":"10.1080\/07328308908048567","volume":"8","author":"Kappes","year":"1989","journal-title":"J. Carbohydr. Chem."},{"key":"10.1074\/jbc.M308523200_bib12","doi-asserted-by":"crossref","first-page":"6804","DOI":"10.1021\/ja960826u","volume":"118","author":"Knapp","year":"1996","journal-title":"J. Am. Chem. Soc."},{"key":"10.1074\/jbc.M308523200_bib13","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1016\/0022-510X(88)90058-5","volume":"87","author":"Rubin","year":"1988","journal-title":"J. Neurol. Sci."},{"key":"10.1074\/jbc.M308523200_bib14","doi-asserted-by":"crossref","first-page":"21386","DOI":"10.1074\/jbc.273.33.21386","volume":"273","author":"Hou","year":"1998","journal-title":"J. Biol. Chem."},{"key":"10.1074\/jbc.M308523200_bib15","series-title":"Enzymes of Lipid Metabolism II","first-page":"779","author":"O'Dowd","year":"1986"},{"key":"10.1074\/jbc.M308523200_bib16","doi-asserted-by":"crossref","first-page":"20799","DOI":"10.1016\/S0021-9258(17)45286-0","volume":"265","author":"Neote","year":"1990","journal-title":"J. Biol. Chem."},{"key":"10.1074\/jbc.M308523200_bib17","first-page":"14","volume":"29","author":"Hoeksema","year":"1977","journal-title":"Am. J. Hum. Genet."},{"key":"10.1074\/jbc.M308523200_bib18","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.M308523200_bib19","doi-asserted-by":"crossref","first-page":"5199","DOI":"10.1074\/jbc.272.8.5199","volume":"272","author":"Glombitza","year":"1997","journal-title":"J. Biol. Chem."},{"key":"10.1074\/jbc.M308523200_bib21","doi-asserted-by":"crossref","first-page":"202","DOI":"10.1006\/prep.2000.1230","volume":"19","author":"Zhao","year":"2000","journal-title":"Protein Expr. Purif."},{"key":"10.1074\/jbc.M308523200_bib22","doi-asserted-by":"crossref","first-page":"25859","DOI":"10.1074\/jbc.R200001200","volume":"277","author":"Kolter","year":"2002","journal-title":"J. Biol. Chem."},{"key":"10.1074\/jbc.M308523200_bib23","doi-asserted-by":"crossref","first-page":"4953","DOI":"10.1016\/S0021-9258(18)83683-3","volume":"264","author":"Jonas","year":"1989","journal-title":"J. Biol. Chem."},{"key":"10.1074\/jbc.M308523200_bib24","doi-asserted-by":"crossref","first-page":"3350","DOI":"10.1016\/S0021-9258(17)43301-1","volume":"259","author":"Proia","year":"1984","journal-title":"J. Biol. Chem."},{"key":"10.1074\/jbc.M308523200_bib25","doi-asserted-by":"crossref","first-page":"1575","DOI":"10.1074\/jbc.M211307200","volume":"278","author":"Loo","year":"2003","journal-title":"J. Biol. Chem."},{"key":"10.1074\/jbc.M308523200_bib26","doi-asserted-by":"crossref","first-page":"284","DOI":"10.1016\/S0387-7604(01)00216-9","volume":"23","author":"Tominaga","year":"2001","journal-title":"Brain Dev."},{"key":"10.1074\/jbc.M308523200_bib27","doi-asserted-by":"crossref","first-page":"112","DOI":"10.1038\/4801","volume":"5","author":"Fan","year":"1999","journal-title":"Nat. Med."},{"key":"10.1074\/jbc.M308523200_bib28","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1056\/NEJM200107053450104","volume":"345","author":"Frustaci","year":"2001","journal-title":"N. Engl. J. Med."},{"key":"10.1074\/jbc.M308523200_bib29","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.M308523200_bib30","doi-asserted-by":"crossref","first-page":"547","DOI":"10.1212\/WNL.47.2.547","volume":"47","author":"De Gasperi","year":"1996","journal-title":"Neurology"},{"key":"10.1074\/jbc.M308523200_bib31","doi-asserted-by":"crossref","first-page":"539","DOI":"10.1212\/WNL.45.3.539","volume":"45","author":"Navon","year":"1995","journal-title":"Neurology"},{"key":"10.1074\/jbc.M308523200_bib32","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1093\/hmg\/2.1.61","volume":"2","author":"Akli","year":"1993","journal-title":"Hum. Mol. Genet."},{"key":"10.1074\/jbc.M308523200_bib33","doi-asserted-by":"crossref","first-page":"142","DOI":"10.1016\/0925-4439(93)90134-M","volume":"1182","author":"Bolhuis","year":"1993","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1074\/jbc.M308523200_bib34","doi-asserted-by":"crossref","first-page":"8198","DOI":"10.1093\/nar\/16.16.8198","volume":"16","author":"Bikker","year":"1988","journal-title":"Nucleic Acids Res."},{"key":"10.1074\/jbc.M308523200_bib35","doi-asserted-by":"crossref","first-page":"129","DOI":"10.1159\/000484748","volume":"5","author":"Fernandes","year":"1997","journal-title":"Eur. J. Hum. Genet."},{"key":"10.1074\/jbc.M308523200_bib36","doi-asserted-by":"crossref","first-page":"428","DOI":"10.1126\/science.276.5311.428","volume":"276","author":"Platt","year":"1997","journal-title":"Science"},{"key":"10.1074\/jbc.M308523200_bib37","doi-asserted-by":"crossref","first-page":"170","DOI":"10.1038\/ng1095-170","volume":"11","author":"Sango","year":"1995","journal-title":"Nat. Genet."},{"key":"10.1074\/jbc.M308523200_bib38","doi-asserted-by":"crossref","first-page":"821","DOI":"10.1016\/S0006-2952(99)00384-6","volume":"59","author":"Andersson","year":"2000","journal-title":"Biochem. Pharmacol."},{"key":"10.1074\/jbc.M308523200_bib20","doi-asserted-by":"crossref","first-page":"719","DOI":"10.1016\/S0040-4039(00)74866-0","volume":"32","author":"Liu","year":"1991","journal-title":"Tetrahedron Lett."},{"key":"10.1074\/jbc.M308523200_bib39","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1042\/bj2360103","volume":"236","author":"Kapur","year":"1986","journal-title":"Biochem. J."},{"key":"10.1074\/jbc.M308523200_bib40","doi-asserted-by":"crossref","first-page":"3055","DOI":"10.1096\/fasebj.5.15.1743438","volume":"5","author":"Elbein","year":"1991","journal-title":"FASEB J."}],"container-title":["Journal of Biological Chemistry"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0021925819640068?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0021925819640068?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1074\/jbc.M308523200","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,1,3]],"date-time":"2022-01-03T01:59:19Z","timestamp":1641175159000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0021925819640068"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2004,4]]},"references-count":40,"journal-issue":{"issue":"14","published-print":{"date-parts":[[2004,4]]}},"alternative-id":["S0021925819640068"],"URL":"https:\/\/doi.org\/10.1074\/jbc.m308523200","relation":{},"ISSN":["0021-9258"],"issn-type":[{"value":"0021-9258","type":"print"}],"subject":[],"published":{"date-parts":[[2004,4]]}}}