{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T23:38:10Z","timestamp":1791329890139,"version":"4.3.1"},"reference-count":23,"publisher":"Wiley","issue":"7-8","license":[{"start":{"date-parts":[[2015,3,24]],"date-time":"2015-03-24T00:00:00Z","timestamp":1427155200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100002738","name":"Assistance Publique H\u00f4pitaux de Paris","doi-asserted-by":"publisher","award":["CIRC07047"],"award-info":[{"award-number":["CIRC07047"]}],"id":[{"id":"10.13039\/501100002738","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Proteomics Clinical Apps"],"published-print":{"date-parts":[[2015,8]]},"abstract":"<jats:sec><jats:title>Purpose<\/jats:title><jats:p>The O\u2010glycan abnormalities accompanying some congenital disorders of glycosylation, namely conserved oligomeric Golgi\u2010congenital disorders of glycosylation (COG\u2010CDGs) and ATP6V0A2\u2010CDGs, are mainly detected using electrophoresis methods applied to circulating apolipoprotein C\u2010III. The objective of this study was to evaluate the reliability of MALDI\u2010TOF MS of apoC\u2010III for the detection and characterization of CDG\u2010associated O\u2010glycan defects.<\/jats:p><\/jats:sec><jats:sec><jats:title>Experimental design<\/jats:title><jats:p>plasmas from CDG\u2010negative, COG\u2010CDG, and ATP6V0A2\u2010CDG patients were analyzed and results were compared to those obtained using 2DE followed by Western blot.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>MALDI\u2010TOF of apoC\u2010III allowed to detect various significant O\u2010glycan abnormalities in CDG\u2010patients with emphasis to COG\u2010CDG. Furthermore, in CDG samples, comparison study between 2DE and MALDI\u2010TOF showed a particular behavior of monosialylated apoC\u2010III in the mass spectrometer that could be related to an abnormal O\u2010glycan structure.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions and clinical relevance<\/jats:title><jats:p>MALDI\u2010TOF MS appears as a powerful technique for the analysis of apoC\u2010III glycoforms for potential routine screening of COG\u2010 and ATP6V0A2\u2010CDGs.<\/jats:p><\/jats:sec>","DOI":"10.1002\/prca.201400187","type":"journal-article","created":{"date-parts":[[2015,1,12]],"date-time":"2015-01-12T13:34:08Z","timestamp":1421069648000},"page":"787-793","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":30,"title":["MALDI\u2010TOF MS applied to apoC\u2010III glycoforms of patients with congenital disorders affecting O\u2010glycosylation. Comparison with two\u2010dimensional electrophoresis"],"prefix":"10.1002","volume":"9","author":[{"given":"St\u00e9phanie","family":"Yen\u2010Nicola\u00ff","sequence":"first","affiliation":[{"name":"Trans\u2010Prot Proteomic facility Universit\u00e9 Paris\u2010Sud  Ch\u00e2tenay\u2010Malabry France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C\u00e9line","family":"Boursier","sequence":"additional","affiliation":[{"name":"Trans\u2010Prot Proteomic facility Universit\u00e9 Paris\u2010Sud  Ch\u00e2tenay\u2010Malabry France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marl\u00e8ne","family":"Rio","sequence":"additional","affiliation":[{"name":"D\u00e9partement de G\u00e9n\u00e9tique H\u00f4pital Necker\u2010Enfants Malades Assistance Publique\u2010H\u00f4pitaux de Paris  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dirk J.","family":"Lefeber","sequence":"additional","affiliation":[{"name":"Laboratory of Genetics 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