{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T21:04:13Z","timestamp":1791147853233,"version":"4.1.0"},"reference-count":28,"publisher":"Elsevier BV","issue":"4","license":[{"start":{"date-parts":[[2008,8,1]],"date-time":"2008-08-01T00:00:00Z","timestamp":1217548800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Molecular Genetics and Metabolism"],"published-print":{"date-parts":[[2008,8]]},"DOI":"10.1016\/j.ymgme.2008.05.001","type":"journal-article","created":{"date-parts":[[2008,6,4]],"date-time":"2008-06-04T09:02:58Z","timestamp":1212570178000},"page":"456-461","source":"Crossref","is-referenced-by-count":57,"title":["Heparin cofactor II\u2013thrombin complex: A biomarker of MPS disease"],"prefix":"10.1016","volume":"94","author":[{"given":"Derrick R.","family":"Randall","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karen E.","family":"Colobong","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Harmony","family":"Hemmelgarn","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Graham B.","family":"Sinclair","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elly","family":"Hetty","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anita","family":"Thomas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olaf A.","family":"Bodamer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Barbara","family":"Volkmar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul M.","family":"Fernhoff","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robin","family":"Casey","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alicia K.","family":"Chan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Grant","family":"Mitchell","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia","family":"Stockler","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Serge","family":"Melancon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tony","family":"Rupar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lorne A.","family":"Clarke","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.ymgme.2008.05.001_bib1","unstructured":"E.F. Neufeld, J. Muenzer, The mucopolysaccharidoses, in: C.R. Scriver et al. (Eds.), Online Metabolic and Molecular Bases of Inherited Disease, 2007 (Chapter 136)."},{"key":"10.1016\/j.ymgme.2008.05.001_bib2","doi-asserted-by":"crossref","first-page":"355","DOI":"10.1007\/s004390050641","article-title":"Incidence of the mucopolysaccharidoses in Northern Ireland","volume":"101","author":"Nelson","year":"1997","journal-title":"Hum. Genet."},{"key":"10.1016\/j.ymgme.2008.05.001_bib3","doi-asserted-by":"crossref","first-page":"310","DOI":"10.1002\/ajmg.a.20314","article-title":"Incidence of the mucopolysaccharidoses in Western Australia","volume":"123","author":"Nelson","year":"2003","journal-title":"Am. J. Med. Genet."},{"key":"10.1016\/j.ymgme.2008.05.001_bib4","doi-asserted-by":"crossref","first-page":"389","DOI":"10.1007\/BF00206770","article-title":"An update on the frequency of mucopolysaccharide syndromes in British Columbia","volume":"85","author":"Lowry","year":"1990","journal-title":"Hum. Genet."},{"key":"10.1016\/j.ymgme.2008.05.001_bib5","doi-asserted-by":"crossref","DOI":"10.1017\/S1462399408000550","article-title":"The mucopolysaccharidoses: a success of molecular medicine","volume":"10","author":"Clarke","year":"2008","journal-title":"Expert Rev. Mol. Med."},{"key":"10.1016\/j.ymgme.2008.05.001_bib6","doi-asserted-by":"crossref","first-page":"286","DOI":"10.1097\/01.GIM.0000078027.83236.49","article-title":"Can mucopolysaccharidosis type I disease severity be predicted based on a patient\u2019s genotype? A comprehensive review of the literature","volume":"5","author":"Terlato","year":"2003","journal-title":"Genet. Med."},{"key":"10.1016\/j.ymgme.2008.05.001_bib7","doi-asserted-by":"crossref","first-page":"82","DOI":"10.1111\/j.1651-2227.2002.tb03116.x","article-title":"Mucopolysaccharidosis type II\u2014genotype\/phenotype aspects","volume":"91","author":"Froissart","year":"2002","journal-title":"Acta Paediatr. Suppl."},{"key":"10.1016\/j.ymgme.2008.05.001_bib8","first-page":"1127","article-title":"Identification, expression, and biochemical characterization of N-acetylgalactosamine-4-sulfatase mutations and relationship with clinical phenotype in MPS-VI patients","volume":"58","author":"Litjens","year":"1996","journal-title":"Am. J. Hum. Genet."},{"key":"10.1016\/j.ymgme.2008.05.001_bib9","doi-asserted-by":"crossref","first-page":"18","DOI":"10.1016\/j.ymgme.2004.09.004","article-title":"Prediction of neuropathology in mucopolysaccharidosis I patients","volume":"84","author":"Fuller","year":"2005","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.ymgme.2008.05.001_bib10","doi-asserted-by":"crossref","first-page":"581","DOI":"10.1016\/j.jpeds.2004.01.046","article-title":"Enzyme replacement therapy for mucopolysaccharidosis I: a randomized, double-blinded, placebo-controlled, multinational study of recombinant human \u03b1-l-iduronidase (Laronidase)","volume":"144","author":"Wraith","year":"2004","journal-title":"J. Pediatr."},{"key":"10.1016\/j.ymgme.2008.05.001_bib11","doi-asserted-by":"crossref","first-page":"465","DOI":"10.1097\/01.gim.0000232477.37660.fb","article-title":"A phase II\/III clinical study of enzyme replacement therapy with idursulfase in mucopolysaccharidosis II (Hunter syndrome)","volume":"8","author":"Muenzer","year":"2006","journal-title":"Genet. Med."},{"key":"10.1016\/j.ymgme.2008.05.001_bib12","doi-asserted-by":"crossref","first-page":"533","DOI":"10.1016\/j.jpeds.2005.12.014","article-title":"MPS VI Phase 3 Study Group, enzyme replacement therapy for mucopolysaccharidosis VI: a phase 3, randomized, double-blinded, placebo-controlled, multinational study of recombinant human N-acetylgalactosamine 4-sulfatase (recombinant human arylsulfatase B or RHASB) and follow-on, open-label extension study","volume":"148","author":"Harmatz","year":"2006","journal-title":"J. Pediatr."},{"key":"10.1016\/j.ymgme.2008.05.001_bib13","doi-asserted-by":"crossref","first-page":"638","DOI":"10.1023\/A:1005525931994","article-title":"Long-term follow-up following bone marrow transplantation for Hunter disease","volume":"22","author":"Vellodi","year":"1999","journal-title":"J. Inherit. Metab. Dis."},{"key":"10.1016\/j.ymgme.2008.05.001_bib14","doi-asserted-by":"crossref","first-page":"849","DOI":"10.1023\/A:1005526628598","article-title":"Bone marrow transplantation in mucopolysaccharidosis type IIIA: a comparison of an early treated patient with his untreated sibling","volume":"22","author":"Sivakumur","year":"1999","journal-title":"J. Inherit. Metab. Dis."},{"key":"10.1016\/j.ymgme.2008.05.001_bib15","doi-asserted-by":"crossref","first-page":"2601","DOI":"10.1182\/blood.V91.7.2601","article-title":"Hurler syndrome: II. Outcome of HLA-genotypically identical sibling and HLA-haploidentical related donor bone marrow transplantation in fifty-four children. The Storage Disease Collaborative Study Group","volume":"91","author":"Peters","year":"1998","journal-title":"Blood"},{"key":"10.1016\/j.ymgme.2008.05.001_bib16","doi-asserted-by":"crossref","first-page":"282","DOI":"10.1006\/mgme.1998.2761","article-title":"Glycosaminoglycan accumulation and excretion in the mucopolysaccharidoses: characterization and basis of a diagnostic test for MPS","volume":"65","author":"Byers","year":"1998","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.ymgme.2008.05.001_bib17","doi-asserted-by":"crossref","first-page":"505","DOI":"10.1016\/S0188-4409(00)00104-1","article-title":"Urinary glycosaminoglycan excretion in healthy subjects and in patients with mucopolysaccharidoses","volume":"31","author":"Gallegos-Arreola","year":"2000","journal-title":"Arch. Med. Res."},{"key":"10.1016\/j.ymgme.2008.05.001_bib18","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1016\/j.cccn.2004.03.015","article-title":"Evaluation of reliability for urine mucopolysaccharidosis screening by dimethylmethylene blue and berry spot tests","volume":"345","author":"Mabe","year":"2004","journal-title":"Clin. Chim. Acta"},{"key":"10.1016\/j.ymgme.2008.05.001_bib19","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1016\/j.ymgme.2006.01.005","article-title":"Heparin cofactor II thrombin complex in MPS I: a biomarker of MPS disease","volume":"88","author":"Randall","year":"2006","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.ymgme.2008.05.001_bib20","doi-asserted-by":"crossref","first-page":"1535","DOI":"10.1046\/j.1538-7836.2003.00305.x","article-title":"Mechanisms of glycosaminoglycan activation of the serpins in hemostasis","volume":"1","author":"Huntington","year":"2003","journal-title":"J. Thromb. Haemost."},{"key":"10.1016\/j.ymgme.2008.05.001_bib21","doi-asserted-by":"crossref","first-page":"6713","DOI":"10.1016\/S0021-9258(18)32276-2","article-title":"Activation of heparin cofactor II by dermatan sulphate","volume":"258","author":"Tollefsen","year":"1983","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.ymgme.2008.05.001_bib22","doi-asserted-by":"crossref","first-page":"4842","DOI":"10.1111\/j.1742-4658.2005.04880.x","article-title":"Control of the coagulation system by serpins. Getting by with a little help from glycosaminoglycans","volume":"272","author":"Pike","year":"2005","journal-title":"FEBS J."},{"key":"10.1016\/j.ymgme.2008.05.001_bib23","doi-asserted-by":"crossref","first-page":"6523","DOI":"10.1074\/jbc.271.11.6523","article-title":"Cellular internalization and degradation of antithrombin III-thrombin, heparin cofactor II\u2013thrombin, and -antitrypsin\u2013trypsin complexes is mediated by the low density lipoprotein receptor-related protein","volume":"271","author":"Kounnas","year":"1996","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.ymgme.2008.05.001_bib24","doi-asserted-by":"crossref","first-page":"224","DOI":"10.1002\/nbm.833","article-title":"Two-dimensional NMR spectroscopy of urinary glycosaminoglycans from patients with different mucopolysaccharidoses","volume":"16","author":"Hochuli","year":"2003","journal-title":"NMR Biomed."},{"key":"10.1016\/j.ymgme.2008.05.001_bib25","doi-asserted-by":"crossref","first-page":"547","DOI":"10.1042\/bj2540547","article-title":"Effect of oversulphated chondroitin and dermatan sulphate upon thrombin and factor Xa inactivation by antithrombin III or heparin cofactor II","volume":"254","author":"Scully","year":"1988","journal-title":"Biochem. J."},{"key":"10.1016\/j.ymgme.2008.05.001_bib26","doi-asserted-by":"crossref","first-page":"733","DOI":"10.1203\/01.PDR.0000141987.69757.DD","article-title":"Disease-specific markers for the mucopolysaccharidoses","volume":"56","author":"Fuller","year":"2004","journal-title":"Pediatr. Res."},{"key":"10.1016\/j.ymgme.2008.05.001_bib27","doi-asserted-by":"crossref","first-page":"6713","DOI":"10.1016\/S0021-9258(18)32276-2","article-title":"Activation of heparin cofactor II by dermatan sulfate","volume":"258","author":"Tollefsen","year":"1983","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.ymgme.2008.05.001_bib28","doi-asserted-by":"crossref","first-page":"4118","DOI":"10.1182\/blood-2007-12-127928","article-title":"Vascular dermatan sulfate regulates the antithrombotic activity of heparin cofactor II","volume":"111","author":"He","year":"2008","journal-title":"Blood"}],"container-title":["Molecular Genetics and Metabolism"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1096719208001443?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1096719208001443?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2021,9,11]],"date-time":"2021-09-11T15:39:31Z","timestamp":1631374771000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1096719208001443"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2008,8]]},"references-count":28,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2008,8]]}},"alternative-id":["S1096719208001443"],"URL":"https:\/\/doi.org\/10.1016\/j.ymgme.2008.05.001","relation":{},"ISSN":["1096-7192"],"issn-type":[{"value":"1096-7192","type":"print"}],"subject":[],"published":{"date-parts":[[2008,8]]}}}