{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T13:49:08Z","timestamp":1790689748340,"version":"4.1.0"},"reference-count":29,"publisher":"Wiley","issue":"18","license":[{"start":{"date-parts":[[2006,7,10]],"date-time":"2006-07-10T00:00:00Z","timestamp":1152489600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["FEBS Letters"],"published-print":{"date-parts":[[2006,8,7]]},"abstract":"<jats:p>\u03b1\u2010Glucosidase (EC 3.2.1.3) is a lysosomal enzyme that hydrolyses \u03b1\u20101,4\u2010 and \u03b1\u20101,6\u2010linkages of glycogen to produce free glucose. A deficiency in \u03b1\u2010glucosidase activity results in glycogen storage disorder type II (GSD II), also called Pompe disease. Here, <jats:sc>d<\/jats:sc>\u2010glucose was shown to be a competitive inhibitor of \u03b1\u2010glucosidase and when added to culture medium at 6.0 g\/L increased the production of this protein by CHO\u2010K1 expression cells and stabilised the enzyme activity. <jats:sc>d<\/jats:sc>\u2010Glucose also prevented \u03b1\u2010glucosidase aggregation\/precipitation and increased protein yield in a modified purification scheme. In fibroblast cells, from adult\u2010onset GSD II patients, <jats:sc>d<\/jats:sc>\u2010glucose increased the residual level of \u03b1\u2010glucosidase activity, suggesting that a structural analogue of <jats:sc>d<\/jats:sc>\u2010glucose may be used for enzyme enhancement therapy.<\/jats:p>","DOI":"10.1016\/j.febslet.2006.06.096","type":"journal-article","created":{"date-parts":[[2006,7,11]],"date-time":"2006-07-11T14:01:21Z","timestamp":1152626481000},"page":"4365-4370","source":"Crossref","is-referenced-by-count":11,"title":["Stabilising normal and mis\u2010sense variant \u03b1\u2010glucosidase"],"prefix":"10.1002","volume":"580","author":[{"given":"Revecca","family":"Kakavanos","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John J.","family":"Hopwood","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Debbie","family":"Lang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter J.","family":"Meikle","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Doug A.","family":"Brooks","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2006,7,10]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1021\/bi00808a017"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1042\/bj0860011"},{"key":"e_1_2_6_4_1","unstructured":"2001 McGraw-Hill New York R. Hirschhorn A.J.J. Reuser C.R. Scriver A.L. Beaudet D. Valle W.S. Sly The Metabolic and Molecular Bases of Inherited Diseases 3389 3420"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/humu.10286"},{"key":"e_1_2_6_6_1","first-page":"132","article-title":"Biochemical, immunological, and cell genetic studies in glycogenesis type II","volume":"30","author":"Reuser A.J.","year":"1978","journal-title":"Am. J. Hum. Genet."},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cccn.2005.05.025"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.113.5.e448"},{"key":"e_1_2_6_9_1","first-page":"169","article-title":"Enzyme replacement therapy for infantile onset Pompe disease: long term follow-up results","volume":"81","author":"Kishnani P.","year":"2004","journal-title":"Mol. Gen. Metab."},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.20019"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2004.10.009"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1432-1033.1995.903_a.x"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.93.1.65"},{"key":"e_1_2_6_14_1","first-page":"613","article-title":"Purification of recombinant human precursor acid alpha-glucosidase","volume":"43","author":"Van Hove J.L.","year":"1997","journal-title":"Biochem. Mol. Biol. Int."},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.192582899"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.2536657100"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1006\/bbrc.1995.2416"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1038\/4801"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M308523200"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1023\/B:BOLI.0000031101.12838.c6"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1159\/000112332"},{"key":"e_1_2_6_22_1","doi-asserted-by":"publisher","DOI":"10.1093\/clinchem\/46.9.1318"},{"key":"e_1_2_6_23_1","doi-asserted-by":"publisher","DOI":"10.1042\/bj3040043"},{"key":"e_1_2_6_24_1","doi-asserted-by":"publisher","DOI":"10.1042\/bj2940657"},{"key":"e_1_2_6_25_1","doi-asserted-by":"publisher","DOI":"10.1016\/0009-8981(78)90517-X"},{"key":"e_1_2_6_26_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1432-1033.1985.tb09159.x"},{"key":"e_1_2_6_27_1","doi-asserted-by":"publisher","DOI":"10.1042\/bj2480755"},{"key":"e_1_2_6_28_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1432-1033.1984.tb08032.x"},{"key":"e_1_2_6_29_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(19)52451-6"},{"key":"e_1_2_6_30_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM200107053450104"}],"container-title":["FEBS Letters"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1016%2Fj.febslet.2006.06.096","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/febs.onlinelibrary.wiley.com\/doi\/pdf\/10.1016\/j.febslet.2006.06.096","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,12]],"date-time":"2023-10-12T13:09:10Z","timestamp":1697116150000},"score":1,"resource":{"primary":{"URL":"https:\/\/febs.onlinelibrary.wiley.com\/doi\/10.1016\/j.febslet.2006.06.096"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2006,7,10]]},"references-count":29,"journal-issue":{"issue":"18","published-print":{"date-parts":[[2006,8,7]]}},"alternative-id":["10.1016\/j.febslet.2006.06.096"],"URL":"https:\/\/doi.org\/10.1016\/j.febslet.2006.06.096","archive":["Portico"],"relation":{},"ISSN":["0014-5793","1873-3468"],"issn-type":[{"value":"0014-5793","type":"print"},{"value":"1873-3468","type":"electronic"}],"subject":[],"published":{"date-parts":[[2006,7,10]]}}}