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Biochemical investigation of suspected oligosaccharidoses has traditionally included thin layer chromatography to detect the presence of disease\u2010specific free oligosaccharides in urine; however, this qualitative method has long been known to have limited sensitivity and specificity. In this unit, a quantitative technique for measuring oligosaccharides utilizing high\u2010performance liquid chromatography\u2013tandem mass spectrometry is described, which provides substantial improvements over other methods, in terms of sensitivity and specificity; moreover, it is relatively inexpensive, accessible, and requires significantly less time, effort, sample volume, and reagents to perform. \u00a9 2024 Wiley Periodicals LLC.<\/jats:p><jats:p><jats:bold>Basic Protocol<\/jats:bold>: Analysis of urinary FOS by HPLC\u2013MS\/MS<\/jats:p>","DOI":"10.1002\/cpz1.70055","type":"journal-article","created":{"date-parts":[[2024,11,22]],"date-time":"2024-11-22T09:26:44Z","timestamp":1732267604000},"source":"Crossref","is-referenced-by-count":1,"title":["Analysis of Free Oligosaccharides in Urine by High\u2010Performance Liquid Chromatography\u2013Tandem Mass Spectrometry"],"prefix":"10.1002","volume":"4","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4249-469X","authenticated-orcid":false,"given":"J. Daniel","family":"Sharer","sequence":"first","affiliation":[{"name":"Biochemical Genetics and Metabolic Disease Laboratory, Department of Genetics, Heersink School of Medicine University of Alabama at Birmingham  Birmingham Alabama"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rongrong","family":"Huang","sequence":"additional","affiliation":[{"name":"Department of Pathology and Immunology, Baylor College of Medicine Harris Health Ben Taub Hospital  Houston Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Timothy","family":"Wood","sequence":"additional","affiliation":[{"name":"Biochemical Genetics Laboratory, Department of Pediatrics University of Colorado Anschutz Medical Campus  Aurora Colorado"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paula","family":"Huffman","sequence":"additional","affiliation":[{"name":"Biochemical Genetics and Metabolic Disease Laboratory, Department of Genetics, Heersink School of Medicine University of Alabama at Birmingham  Birmingham Alabama"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yang","family":"Yan","sequence":"additional","affiliation":[{"name":"Biochemical Genetics and Metabolic Disease Laboratory, Department of Genetics, Heersink School of Medicine University of Alabama at Birmingham  Birmingham Alabama"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chelsea","family":"Zimmerman","sequence":"additional","affiliation":[{"name":"Biochemical Laboratory Oklahoma University Health Science Center  Oklahoma City Oklahoma"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Pollard","sequence":"additional","affiliation":[{"name":"Biochemical Genetics Laboratory Greenwood Genetic Center  Greenwood South Carolina"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,11,22]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1186\/1750\u20101172\u20109\u201019"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00018\u2010013\u20101538\u20103"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00216\u2010012\u20105968\u20109"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1373\/clinchem.2018.289645"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1044\u20100305(00)00163\u2010X"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2021.08.006"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2019.04.008"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/rcm.7860"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ab.2005.06.042"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023\u2010020\u201001662\u20108"},{"key":"e_1_2_6_12_1","first-page":"219","volume-title":"Techniques in diagnostic human biochemical genetics: A laboratory manual","author":"Sewell A. 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