{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,21]],"date-time":"2026-09-21T22:17:48Z","timestamp":1790029068828,"version":"4.0.1"},"reference-count":25,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2008,12,31]],"date-time":"2008-12-31T00:00:00Z","timestamp":1230681600000},"content-version":"vor","delay-in-days":3927,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Deutsche Forschungs Gemeinschaft","award":["SI3 284"],"award-info":[{"award-number":["SI3 284"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Protein Science"],"published-print":{"date-parts":[[1998,4]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Lysosomal degradation of ganglioside GM2 by hexosaminidase A requires the presence of a small, non\u2010enzymatic cofactor, the GM2\u2010activator protein (GM2AP). Lack of functional protein leads to the AB variant of GM2\u2010gangliosidosis, a fatal lysosomal storage disease.<\/jats:p>\n                  <jats:p>\n                    Although its possible mode of action and functional domains have been discussed frequently in the past, no structural information about GM2AP is available so far. Here, we determine the complete disulfide bond pattern of the protein. Two of the four disulfide bonds present in the protein were open to classical determination by enzymatic cleavage and mass spectrometry. The direct localization of the remaining two bonds was impeded by the close vicinity of cysteines 136 and 138. We determined the arrangement of these disulfide bonds by MALDI\u2010PSD analysis of disulfide linked peptides and by partial reduction, cyanylation and fragmentation in basic solution, as described recently (Wu F, Watson JT, 1997,\n                    <jats:italic>Protein Sci 6<\/jats:italic>\n                    :391\u2010398).\n                  <\/jats:p>","DOI":"10.1002\/pro.5560070421","type":"journal-article","created":{"date-parts":[[2010,7,12]],"date-time":"2010-07-12T03:18:12Z","timestamp":1278904692000},"page":"1039-1045","source":"Crossref","is-referenced-by-count":26,"title":["Complete localization of disulfide bonds in GM2 activator protein"],"prefix":"10.1002","volume":"7","author":[{"given":"Christina G.","family":"Sch\u00fctte","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thorsten","family":"Lemm","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Konrad","family":"Sandhoff","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gereon J.","family":"Glombitza","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2008,12,31]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-1809.1985.tb01674.x"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.75.8.3979"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1515\/bchm3.1988.369.1.317"},{"key":"e_1_2_1_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1432-1033.1990.tb19280.x"},{"key":"e_1_2_1_6_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.272.8.5199"},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/pro.5560060619"},{"key":"e_1_2_1_8_1","doi-asserted-by":"publisher","DOI":"10.1038\/nbt1188-1321"},{"key":"e_1_2_1_9_1","first-page":"137","article-title":"Sequencing of peptides in a time\u2010of\u2010flight mass spectrometer: Evaluation of postsource decay following matrix\u2010assisted laser desorption ionization (MALD1)","volume":"86","author":"Kaufmann R","year":"1994","journal-title":"Int J Mass Spectrom Ion Proc"},{"key":"e_1_2_1_10_1","doi-asserted-by":"publisher","DOI":"10.1042\/bj2920571"},{"key":"e_1_2_1_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/0014-5793(91)81084-L"},{"key":"e_1_2_1_12_1","doi-asserted-by":"crossref","first-page":"807","DOI":"10.1042\/bj2820807","article-title":"Evidence for two cDNA clones encoding human GM2\u2010activator protein","volume":"282","author":"Nagarajan S","year":"1992","journal-title":"Biochem J"},{"key":"e_1_2_1_13_1","doi-asserted-by":"publisher","DOI":"10.1093\/oxfordjournals.jbchem.a122629"},{"key":"e_1_2_1_14_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.2842863"},{"key":"e_1_2_1_15_1","doi-asserted-by":"publisher","DOI":"10.1021\/ac00093a030"},{"key":"e_1_2_1_16_1","first-page":"2427","volume-title":"The metabolic bases of inherited disease","author":"Sandhoff K","year":"1992"},{"key":"e_1_2_1_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/0014-5793(91)81211-P"},{"key":"e_1_2_1_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0167-4838(97)00002-2"},{"key":"e_1_2_1_19_1","doi-asserted-by":"publisher","DOI":"10.1002\/pro.5560020302"},{"key":"e_1_2_1_20_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.270.17.9953"},{"key":"e_1_2_1_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/0025-5564(94)90004-3"},{"key":"e_1_2_1_22_1","doi-asserted-by":"publisher","DOI":"10.1002\/pro.5560060215"},{"key":"e_1_2_1_23_1","first-page":"16276","article-title":"Expression and specificity of human GM2 activator protein","volume":"169","author":"Wu Y\u2010Y","year":"1996","journal-title":"J Biol Chem"},{"key":"e_1_2_1_24_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.271.18.10611"},{"key":"e_1_2_1_25_1","doi-asserted-by":"publisher","DOI":"10.1016\/0006-291X(91)90671-S"},{"key":"e_1_2_1_26_1","first-page":"1046","article-title":"A Cys138\u2010to Arg substitution in the GM2 activator protein is associated with the AB variant form of GM2 gangliosidosis","volume":"50","author":"Xie B","year":"1992","journal-title":"Ann J Hum Genet"}],"container-title":["Protein Science"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fpro.5560070421","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fpro.5560070421","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/pro.5560070421","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,28]],"date-time":"2023-10-28T13:40:34Z","timestamp":1698500434000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/pro.5560070421"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1998,4]]},"references-count":25,"journal-issue":{"issue":"4","published-print":{"date-parts":[[1998,4]]}},"alternative-id":["10.1002\/pro.5560070421"],"URL":"https:\/\/doi.org\/10.1002\/pro.5560070421","archive":["Portico"],"relation":{},"ISSN":["0961-8368","1469-896X"],"issn-type":[{"value":"0961-8368","type":"print"},{"value":"1469-896X","type":"electronic"}],"subject":[],"published":{"date-parts":[[1998,4]]}}}