{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,16]],"date-time":"2026-06-16T06:44:05Z","timestamp":1781592245409,"version":"3.54.5"},"reference-count":29,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2025,2,24]],"date-time":"2025-02-24T00:00:00Z","timestamp":1740355200000},"content-version":"vor","delay-in-days":23,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2025,2,1]],"date-time":"2025-02-01T00:00:00Z","timestamp":1738368000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Current Protocols"],"published-print":{"date-parts":[[2025,2]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Mucopolysaccharidoses (MPSs) are complex lysosomal diseases that result in the accumulation of glycosaminoglycans (GAGs) in urine, blood, and tissues. Lysosomal enzymes responsible for GAG degradation are defective in MPSs. GAGs including chondroitin sulfate (CS), dermatan sulfate (DS), heparan sulfate (HS), and keratan sulfate (KS) are biomarkers for MPSs. This article describes a stable isotope dilution\u2010tandem mass spectrometric method for quantifying CS, DS, and HS in urine samples. The GAGs are methanolyzed to uronic\/iduronic acid\u2010\n                    <jats:italic>N<\/jats:italic>\n                    \u2010acetylhexosamine or uronic\/iduronic acid\u2010\n                    <jats:italic>N<\/jats:italic>\n                    \u2010glucosamine dimers and mixed with internal standards derived from deuteriomethanolysis of GAG standards. Specific dimers derived from HS, DS, and CS are separated by ultra\u2010performance liquid chromatography (UPLC) and analyzed by electrospray ionization (ESI) tandem mass spectrometry (MS\/MS) using selected reaction monitoring for each targeted GAG product and its corresponding internal standard. This UPLC\u2010MS\/MS GAG assay is useful for identifying patients with MPS types I, II, III, VI, and VII. \u00a9 2025 Wiley Periodicals LLC.\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:bold>Basic Protocol<\/jats:bold>\n                    : Urinary GAG analysis by ESI\u2010MS\/MS\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:bold>Support Protocol 1<\/jats:bold>\n                    : Prepare calibration samples\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:bold>Support Protocol 2<\/jats:bold>\n                    : Preparation of stable\u2010isotope\u2010labeled internal standards\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:bold>Support Protocol 3<\/jats:bold>\n                    : Preparation of quality controls for GAG analysis in urine\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:bold>Support Protocol 4<\/jats:bold>\n                    : Optimization of methanolysis time\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:bold>Support Protocol 5<\/jats:bold>\n                    : Measurement of methanolic HCl concentration\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:bold>Support Protocol 6<\/jats:bold>\n                    : Preparation of working methanolic HCl solution (1.1 M)\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:bold>Support Protocol 7<\/jats:bold>\n                    : Dilution of prepared urine sample\n                  <\/jats:p>","DOI":"10.1002\/cpz1.70110","type":"journal-article","created":{"date-parts":[[2025,2,27]],"date-time":"2025-02-27T12:58:16Z","timestamp":1740661096000},"source":"Crossref","is-referenced-by-count":3,"title":["Quantification of Glycosaminoglycans in Urine by Isotope Dilution Liquid Chromatography\u2010Electrospray Ionization Tandem Mass Spectrometry"],"prefix":"10.1002","volume":"5","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8869-4531","authenticated-orcid":false,"given":"Haoyue","family":"Zhang","sequence":"first","affiliation":[{"name":"Biochemical Genetics Laboratory Duke University Health System  Durham North Carolina"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James","family":"Beasley","sequence":"additional","affiliation":[{"name":"Biochemical Genetics Laboratory Duke University Health System  Durham North Carolina"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iskren","family":"Menkovic","sequence":"additional","affiliation":[{"name":"Division of Medical Genetics, Department of Pediatrics Duke University Medical Center  Durham North Carolina"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ashlee","family":"Stiles","sequence":"additional","affiliation":[{"name":"Biochemical Genetics Laboratory Duke University Health System  Durham North Carolina"},{"name":"Division of Medical Genetics, Department of Pediatrics Duke University Medical Center  Durham North Carolina"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David S.","family":"Millington","sequence":"additional","affiliation":[{"name":"Biochemical Genetics Laboratory Duke University Health System  Durham North Carolina"},{"name":"Division of Medical Genetics, Department of Pediatrics Duke University Medical Center  Durham North Carolina"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah P.","family":"Young","sequence":"additional","affiliation":[{"name":"Biochemical Genetics Laboratory Duke University Health System  Durham North Carolina"},{"name":"Division of Medical Genetics, Department of Pediatrics Duke University Medical Center  Durham North 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