{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,30]],"date-time":"2026-05-30T02:59:41Z","timestamp":1780109981352,"version":"3.54.0"},"reference-count":46,"publisher":"Elsevier BV","issue":"3","license":[{"start":{"date-parts":[[2005,12,1]],"date-time":"2005-12-01T00:00:00Z","timestamp":1133395200000},"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":["Neurobiology of Disease"],"published-print":{"date-parts":[[2005,12]]},"DOI":"10.1016\/j.nbd.2005.05.009","type":"journal-article","created":{"date-parts":[[2005,6,28]],"date-time":"2005-06-28T17:13:09Z","timestamp":1119978789000},"page":"738-743","source":"Crossref","is-referenced-by-count":17,"title":["The metabolism of glycosaminoglycans is impaired in prion diseases"],"prefix":"10.1016","volume":"20","author":[{"given":"Tehila","family":"Mayer-Sonnenfeld","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marsha","family":"Zeigler","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michele","family":"Halimi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yael","family":"Dayan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian","family":"Herzog","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Corinne I.","family":"Lasmezas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ruth","family":"Gabizon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.nbd.2005.05.009_bib1","doi-asserted-by":"crossref","first-page":"449","DOI":"10.1016\/0009-9120(87)90013-0","article-title":"Urinary excretion of glycosaminoglycans in urological disease","volume":"20","author":"Baggio","year":"1987","journal-title":"Clin. Biochem."},{"key":"10.1016\/j.nbd.2005.05.009_bib2","doi-asserted-by":"crossref","first-page":"481","DOI":"10.1046\/j.1471-4159.2002.01192.x","article-title":"The neuropathogenic contributions of lysosomal dysfunction","volume":"83","author":"Bahr","year":"2002","journal-title":"J. Neurochem."},{"key":"10.1016\/j.nbd.2005.05.009_bib3","doi-asserted-by":"crossref","first-page":"8462","DOI":"10.1128\/JVI.77.15.8462-8469.2003","article-title":"Evaluation of quinacrine treatment for prion diseases","volume":"77","author":"Barret","year":"2003","journal-title":"J. Virol."},{"key":"10.1016\/j.nbd.2005.05.009_bib4","doi-asserted-by":"crossref","first-page":"40041","DOI":"10.1074\/jbc.M301152200","article-title":"Cellular heparan sulfate participates in the metabolism of prions","volume":"278","author":"Ben-Zaken","year":"2003","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2005.05.009_bib5","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1093\/bmb\/66.1.121","article-title":"Neuropathology of prion diseases","volume":"66","author":"Budka","year":"2003","journal-title":"Br. Med. Bull."},{"issue":"2","key":"10.1016\/j.nbd.2005.05.009_bib6","doi-asserted-by":"crossref","first-page":"643","DOI":"10.1128\/JVI.67.2.643-650.1993","article-title":"Sulfated polyanion inhibition of scrapie-associated PrP accumulation in cultured cells","volume":"67","author":"Caughey","year":"1993","journal-title":"J. Virol."},{"key":"10.1016\/j.nbd.2005.05.009_bib7","series-title":"Techniques in Diagnostic Human Biochemical Genetics","first-page":"77","article-title":"Screening for mucopolysaccharidoses by analysis of urinary glycosaminoglycans","author":"Dembure","year":"1991"},{"key":"10.1016\/j.nbd.2005.05.009_bib8","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1016\/0009-9120(90)90561-8","article-title":"Selective urinary screening for mucopolysaccharidoses","volume":"23","author":"Dembure","year":"1990","journal-title":"Clin. Biochem."},{"key":"10.1016\/j.nbd.2005.05.009_bib9","doi-asserted-by":"crossref","first-page":"1323","DOI":"10.1016\/S0196-9781(02)00068-2","article-title":"Glycosaminoglycans and beta-amyloid, prion and tau peptides in neurodegenerative diseases","volume":"23","author":"Diaz-Nido","year":"2002","journal-title":"Peptides"},{"key":"10.1016\/j.nbd.2005.05.009_bib10","doi-asserted-by":"crossref","first-page":"4894","DOI":"10.1128\/JVI.74.10.4894-4897.2000","article-title":"Lysosomotropic agents and cysteine protease inhibitors inhibit scrapie-associated prion protein accumulation","volume":"74","author":"Doh-Ura","year":"2000","journal-title":"J. Virol."},{"key":"10.1016\/j.nbd.2005.05.009_bib11","doi-asserted-by":"crossref","first-page":"4999","DOI":"10.1128\/JVI.78.10.4999-5006.2004","article-title":"Treatment of transmissible spongiform encephalopathy by intraventricular drug infusion in animal models","volume":"78","author":"Doh-ura","year":"2004","journal-title":"J. Virol."},{"issue":"Pt. 8","key":"10.1016\/j.nbd.2005.05.009_bib12","doi-asserted-by":"crossref","first-page":"1325","DOI":"10.1099\/0022-1317-65-8-1325","article-title":"Dextran sulphate 500 delays and prevents mouse scrapie by impairment of agent replication in spleen","volume":"65","author":"Ehlers","year":"1984","journal-title":"J. Gen. Virol."},{"key":"10.1016\/j.nbd.2005.05.009_bib13","doi-asserted-by":"crossref","first-page":"1749","DOI":"10.1096\/fj.03-1436fje","article-title":"Amyloidogenesis recapitulated in cell culture: a peptide inhibitor provides direct evidence for the role of heparan sulfate and suggests a new treatment strategy","volume":"18","author":"Elimova","year":"2004","journal-title":"FASEB J."},{"key":"10.1016\/j.nbd.2005.05.009_bib14","doi-asserted-by":"crossref","first-page":"1317","DOI":"10.1016\/S0306-3623(96)00150-4","article-title":"Drug-induced lysosomal storage of sulphated glycosaminoglycans","volume":"27","author":"Fischer","year":"1996","journal-title":"Gen. Pharmacol."},{"key":"10.1016\/j.nbd.2005.05.009_bib15","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1002\/jcp.1041570215","article-title":"Heparin-like molecules bind differentially to prion-proteins and change their intracellular metabolic fate","volume":"157","author":"Gabizon","year":"1993","journal-title":"J. Cell. Physiol."},{"key":"10.1016\/j.nbd.2005.05.009_bib16","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.nbd.2005.05.009_bib17","first-page":"1332","article-title":"Hydroxyproline and acid glycosaminoglycans in acute cerebral circulatory disorders","volume":"79","author":"Gusev","year":"1979","journal-title":"Z. Nevropatol. Psihiatr. S.S. Korsakova"},{"issue":"17","key":"10.1016\/j.nbd.2005.05.009_bib18","doi-asserted-by":"crossref","first-page":"17057","DOI":"10.1074\/jbc.M411314200","article-title":"PrPSc incorporation to cells requires endogenous glycosaminoglycan expression","volume":"280","author":"Hijazi","year":"2005","journal-title":"J. Biol. Chem."},{"issue":"17","key":"10.1016\/j.nbd.2005.05.009_bib19","doi-asserted-by":"crossref","first-page":"17062","DOI":"10.1074\/jbc.M500122200","article-title":"Heparan sulfate is a cellular receptor for purified infectious prions","volume":"280","author":"Horonchik","year":"2005","journal-title":"J. Biol. Chem."},{"issue":"16","key":"10.1016\/j.nbd.2005.05.009_bib20","doi-asserted-by":"crossref","first-page":"1091","DOI":"10.1056\/NEJM199104183241604","article-title":"Mutation of the prion protein in Libyan Jews with Creutzfeldt\u2013Jakob disease","volume":"324","author":"Hsiao","year":"1991","journal-title":"N. Engl. J. Med."},{"key":"10.1016\/j.nbd.2005.05.009_bib21","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1016\/0009-8981(85)90317-1","article-title":"Glycosaminoglycan excretion in random samples of urine","volume":"151","author":"Huang","year":"1985","journal-title":"Clin. Chim. Acta"},{"key":"10.1016\/j.nbd.2005.05.009_bib22","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1016\/0009-8981(85)90318-3","article-title":"Screening test for urinary glycosaminoglycans and differentiation of various mucopolysaccharidoses","volume":"151","author":"Huang","year":"1985","journal-title":"Clin. Chim. Acta"},{"key":"10.1016\/j.nbd.2005.05.009_bib23","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1016\/S0378-4347(00)83875-X","article-title":"Methods for analysis of urinary glycosaminoglycans","volume":"429","author":"Kodama","year":"1988","journal-title":"J. Chromatogr."},{"key":"10.1016\/j.nbd.2005.05.009_bib24","doi-asserted-by":"crossref","first-page":"411","DOI":"10.1128\/JVI.74.1.411-417.2000","article-title":"Upregulation of the genes encoding lysosomal hydrolases, a perforin-like protein, and peroxidases in the brains of mice affected with an experimental prion disease","volume":"74","author":"Kopacek","year":"2000","journal-title":"J. Virol."},{"key":"10.1016\/j.nbd.2005.05.009_bib25","doi-asserted-by":"crossref","first-page":"9836","DOI":"10.1073\/pnas.161274798","article-title":"Acridine and phenothiazine derivatives as pharmacotherapeutics for prion disease","volume":"98","author":"Korth","year":"2001","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"issue":"Pt. 3","key":"10.1016\/j.nbd.2005.05.009_bib26","doi-asserted-by":"crossref","first-page":"661","DOI":"10.1099\/0022-1317-73-3-661","article-title":"Sulphate polyanions prolong the incubation period of scrapie-infected hamsters","volume":"73","author":"Ladogana","year":"1992","journal-title":"J. Gen. Virol."},{"key":"10.1016\/j.nbd.2005.05.009_bib27","doi-asserted-by":"crossref","first-page":"333","DOI":"10.1002\/path.1711660404","article-title":"Lysosomes as key organelles in the pathogenesis of prion encephalopathies","volume":"166","author":"Laszlo","year":"1992","journal-title":"J. Pathol."},{"key":"10.1016\/j.nbd.2005.05.009_bib28","doi-asserted-by":"crossref","first-page":"673","DOI":"10.1126\/science.1100195","article-title":"Synthetic mammalian prions","volume":"305","author":"Legname","year":"2004","journal-title":"Science"},{"key":"10.1016\/j.nbd.2005.05.009_bib29","doi-asserted-by":"crossref","first-page":"620","DOI":"10.1523\/JNEUROSCI.4303-03.2004","article-title":"Neurons and astrocytes respond to prion infection by inducing microglia recruitment","volume":"24","author":"Marella","year":"2004","journal-title":"J. Neurosci."},{"key":"10.1016\/j.nbd.2005.05.009_bib30","doi-asserted-by":"crossref","DOI":"10.1074\/jbc.M410966200","article-title":"Pathological prion protein exposure switches on neuronal MAP-kinase pathway resulting in microglia recruitment","author":"Marella","year":"2004","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2005.05.009_bib31","first-page":"622","article-title":"Ultrastructural localization of scrapie prion proteins in cytoplasmic vesicles of infected cultured cells","volume":"65","author":"McKinley","year":"1991","journal-title":"Lab. Invest."},{"key":"10.1016\/j.nbd.2005.05.009_bib32","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1006\/abio.1993.1098","article-title":"Combined alcian blue and silver staining of subnanogram quantities of proteoglycans and glycosaminoglycans in sodium dodecyl sulfate-polyacrylamide gels","volume":"209","author":"Moller","year":"1993","journal-title":"Anal. Biochem."},{"key":"10.1016\/j.nbd.2005.05.009_bib33","first-page":"81","article-title":"The biochemical basis for mucopolysaccharidoses and mucolipidoses","volume":"10","author":"Neufeld","year":"1974","journal-title":"Prog. Med. Genet."},{"key":"10.1016\/j.nbd.2005.05.009_bib34","series-title":"The Mucopolysaccharidoses. The Metabolic and Molecular Bases of Inherited Diseases","first-page":"3421","author":"Neufeld","year":"2001"},{"key":"10.1016\/j.nbd.2005.05.009_bib35","doi-asserted-by":"crossref","first-page":"1902","DOI":"10.1073\/pnas.252784899","article-title":"Activated microglia in cortex of mouse models of mucopolysaccharidoses I and IIIB","volume":"100","author":"Ohmi","year":"2003","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"issue":"47","key":"10.1016\/j.nbd.2005.05.009_bib36","doi-asserted-by":"crossref","first-page":"48817","DOI":"10.1074\/jbc.M409107200","article-title":"Immunoglobulins in urine of hamsters with scrapie","volume":"279","author":"Serban","year":"2004","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2005.05.009_bib37","doi-asserted-by":"crossref","first-page":"478","DOI":"10.1016\/j.ymthe.2004.05.029","article-title":"Widespread correction of lysosomal storage following intrahepatic injection of a recombinant adeno-associated virus in the adult MPS VII mouse","volume":"10","author":"Sferra","year":"2004","journal-title":"Mol. Ther."},{"issue":"5","key":"10.1016\/j.nbd.2005.05.009_bib38","first-page":"601","article-title":"Immunolocalization of heparan sulfate proteoglycans to the prion protein amyloid plaques of Gerstmann\u2013Straussler syndrome, Creutzfeldt\u2013Jakob disease and scrapie","volume":"63","author":"Snow","year":"1990","journal-title":"Lab. Invest."},{"key":"10.1016\/j.nbd.2005.05.009_bib39","doi-asserted-by":"crossref","first-page":"274","DOI":"10.1016\/S0753-3322(99)80098-5","article-title":"Urinary glycosaminoglycans in recurrent urinary tract infections in kidney transplant patients","volume":"53","author":"Stabellini","year":"1999","journal-title":"Biomed. Pharmacother."},{"key":"10.1016\/j.nbd.2005.05.009_bib40","doi-asserted-by":"crossref","first-page":"10954","DOI":"10.1073\/pnas.97.20.10954","article-title":"Microglial activation precedes acute neurodegeneration in Sandhoff disease and is suppressed by bone marrow transplantation","volume":"97","author":"Wada","year":"2000","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.nbd.2005.05.009_bib41","doi-asserted-by":"crossref","first-page":"1577","DOI":"10.1016\/S0022-5347(05)67682-3","article-title":"The association of elevated urinary total to sulfated glycosaminoglycan ratio and high molecular mass hyaluronic acid with interstitial cystitis","volume":"163","author":"Wei","year":"2000","journal-title":"J. Urol."},{"key":"10.1016\/j.nbd.2005.05.009_bib42","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1093\/bmb\/66.1.43","article-title":"PrP knock-out and PrP transgenic mice in prion research","volume":"66","author":"Weissmann","year":"2003","journal-title":"Br. Med. Bull."},{"key":"10.1016\/j.nbd.2005.05.009_bib43","series-title":"Techniques in Diagnostic Human Biochemical","first-page":"587","article-title":"Screening for lysosomal disorders","author":"Wenger","year":"1991"},{"key":"10.1016\/j.nbd.2005.05.009_bib44","doi-asserted-by":"crossref","first-page":"2279","DOI":"10.1099\/vir.0.19153-0","article-title":"Up-regulation of cathepsin B and cathepsin L activities in scrapie-infected mouse Neuro2a cells","volume":"84","author":"Zhang","year":"2003","journal-title":"J. Gen. Virol."},{"key":"10.1016\/j.nbd.2005.05.009_bib45","doi-asserted-by":"crossref","first-page":"6101","DOI":"10.1073\/pnas.93.12.6101","article-title":"The molecular basis of Sanfilippo syndrome type B","volume":"93","author":"Zhao","year":"1996","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.nbd.2005.05.009_bib46","doi-asserted-by":"crossref","first-page":"286","DOI":"10.1016\/j.ymgme.2004.06.004","article-title":"Retrovirally transduced bone marrow has a therapeutic effect on brain in the mouse model of mucopolysaccharidosis IIIB","volume":"82","author":"Zheng","year":"2004","journal-title":"Mol. Genet. Metab."}],"container-title":["Neurobiology of Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0969996105001440?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0969996105001440?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2020,4,8]],"date-time":"2020-04-08T01:21:30Z","timestamp":1586308890000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0969996105001440"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2005,12]]},"references-count":46,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2005,12]]}},"alternative-id":["S0969996105001440"],"URL":"https:\/\/doi.org\/10.1016\/j.nbd.2005.05.009","relation":{},"ISSN":["0969-9961"],"issn-type":[{"value":"0969-9961","type":"print"}],"subject":[],"published":{"date-parts":[[2005,12]]}}}