{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,28]],"date-time":"2026-10-28T09:35:06Z","timestamp":1793180106456,"version":"4.5.0"},"reference-count":122,"publisher":"Elsevier BV","issue":"4","license":[{"start":{"date-parts":[[2018,12,1]],"date-time":"2018-12-01T00:00:00Z","timestamp":1543622400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2018,12,1]],"date-time":"2018-12-01T00:00:00Z","timestamp":1543622400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2018,9,4]],"date-time":"2018-09-04T00:00:00Z","timestamp":1536019200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"name":"Orchard Therapeutics","id":[{"id":"https:\/\/ror.org\/050adjk75","id-type":"ROR","asserted-by":"crossref"}]},{"name":"Foundation Puglia"},{"DOI":"10.13039\/100008484","name":"BioMarin Pharmaceutical Inc","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100008484","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Molecular Genetics and Metabolism"],"published-print":{"date-parts":[[2018,12]]},"DOI":"10.1016\/j.ymgme.2018.08.003","type":"journal-article","created":{"date-parts":[[2018,8,10]],"date-time":"2018-08-10T12:36:12Z","timestamp":1533904572000},"page":"322-331","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":99,"title":["Anatomical changes and pathophysiology of the brain in mucopolysaccharidosis disorders"],"prefix":"10.1016","volume":"125","author":[{"given":"Brian W.","family":"Bigger","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David J.","family":"Begley","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniela","family":"Virgintino","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexey V.","family":"Pshezhetsky","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.ymgme.2018.08.003_bb0005","series-title":"The Metabolic and Molecular Bases of Inherited Disease","first-page":"3421","article-title":"The mucopolysaccharidoses","author":"Neufeld","year":"2001"},{"issue":"Suppl. 5","key":"10.1016\/j.ymgme.2018.08.003_bb0010","doi-asserted-by":"crossref","first-page":"v4","DOI":"10.1093\/rheumatology\/ker394","article-title":"Overview of the mucopolysaccharidoses","volume":"50","author":"Muenzer","year":"2011","journal-title":"Rheumatology (Oxford)"},{"key":"10.1016\/j.ymgme.2018.08.003_bb0015","doi-asserted-by":"crossref","first-page":"605","DOI":"10.1016\/j.mehy.2010.07.044","article-title":"Why are behaviors of children suffering from various neuronopathic types of mucopolysaccharidoses different?","volume":"75","author":"W\u0119grzyn","year":"2010","journal-title":"Med. Hypotheses"},{"key":"10.1016\/j.ymgme.2018.08.003_bb0020","doi-asserted-by":"crossref","first-page":"1343","DOI":"10.1007\/s11011-015-9684-y","article-title":"Mental retardation in mucopolysaccharidoses correlates with high molecular weight urinary heparan sulphate derived glucosamine","volume":"30","author":"Coppa","year":"2015","journal-title":"Metab. Brain Dis."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0025","doi-asserted-by":"crossref","first-page":"5","DOI":"10.3174\/ajnr.A2832","article-title":"Brain and spinal MR imaging findings in mucopolysaccharidoses: a review AJNR","volume":"34","author":"Zafeiriou","year":"2013","journal-title":"Am. J. Neuroradiol."},{"issue":"5 Suppl","key":"10.1016\/j.ymgme.2018.08.003_bb0030","doi-asserted-by":"crossref","first-page":"S27","DOI":"10.1016\/j.jpeds.2004.01.052","article-title":"The mucopolysaccharidoses: a heterogeneous group of disorders with variable pediatric presentations","volume":"144","author":"Muenzer","year":"2004","journal-title":"J. Pediatr."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0035","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1136\/adc.73.1.77","article-title":"Behaviour in mucopolysaccharide disorders","volume":"73","author":"Bax","year":"1995","journal-title":"Arch. Dis. Child."},{"issue":"Suppl. 1","key":"10.1016\/j.ymgme.2018.08.003_bb0040","first-page":"133","article-title":"Mucopolysaccharidosis III (Sanfilippo Syndrome)- disease presentation and experimental therapies","volume":"12","author":"Gilkes","year":"2014","journal-title":"Pediatr. Endocrinol. Rev."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0045","doi-asserted-by":"crossref","first-page":"e1258","DOI":"10.1542\/peds.2010-1274","article-title":"Natural progression of neurological disease in mucopolysaccharidosis type II","volume":"127","author":"Holt","year":"2011","journal-title":"Pediatrics"},{"key":"10.1016\/j.ymgme.2018.08.003_bb0050","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1016\/j.ymgme.2015.06.002","article-title":"Neurocognition across the spectrum of mucopolysaccharidosis type I: age, severity, and treatment","volume":"116","author":"Shapiro","year":"2015","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0055","doi-asserted-by":"crossref","first-page":"413","DOI":"10.1007\/BF00710053","article-title":"Neuropsychological outcomes of several storage diseases with and without bone marrow transplantation","volume":"18","author":"Shapiro","year":"1995","journal-title":"J. Inherit. Metab. Dis."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0060","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.ymgme.2017.08.009","article-title":"Developmental and behavioral aspects of mucopolysaccharidoses with brain manifestations - neurological signs and symptoms","volume":"122S","author":"Shapiro","year":"2017","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0065","first-page":"32","article-title":"A longitudinal study of emotional adjustment, quality of life and adaptive function in attenuated MPS II","volume":"7","author":"Shapiro","year":"2016","journal-title":"Mol. Genet. Metab. Rep."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0070","doi-asserted-by":"crossref","first-page":"170","DOI":"10.1016\/j.ymgme.2014.12.299","article-title":"Cognitive, medical, and neuroimaging characteristics of attenuated mucopolysaccharidosis type II","volume":"114","author":"Yund","year":"2015","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0075","doi-asserted-by":"crossref","first-page":"759","DOI":"10.1038\/gim.2014.25","article-title":"The natural history of MPS I: global perspectives from the MPS I Registry","volume":"16","author":"Beck","year":"2014","journal-title":"Genet. Med."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0080","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1007\/BF00690379","article-title":"Mucopolysaccharidosis type I, II, IIIA and V. Pathological and biochemical abnormalities in the neural and mesenchymal elements of the brain","volume":"39","author":"Dekaban","year":"1977","journal-title":"Acta Neuropathol."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0085","first-page":"132","article-title":"Mucopolysaccharidosis VII: postmortem biochemical and pathological findings in a young adult with \u03b2-glucuronidase deficiency","volume":"7","author":"Vogler","year":"1994","journal-title":"Mod. Pathol."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0090","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1016\/j.ymgme.2017.09.006","article-title":"Quantitative neuroimaging in mucopolysaccharidoses clinical trials","volume":"122S","author":"Nestrasil","year":"2017","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0095","doi-asserted-by":"crossref","first-page":"1029","DOI":"10.3174\/ajnr.A0510","article-title":"Correlation of MR imaging and MR spectroscopy findings with cognitive impairment in mucopolysaccharidosis II AJNR","volume":"28","author":"Vedolin","year":"2007","journal-title":"Am. J. Neuroradiol."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0100","doi-asserted-by":"crossref","first-page":"917","DOI":"10.1212\/01.wnl.0000269782.80107.fe","article-title":"Brain MRI in mucopolysaccharidosis: effect of aging and correlation with biochemical findings","volume":"69","author":"Vedolin","year":"2007","journal-title":"Neurology"},{"key":"10.1016\/j.ymgme.2018.08.003_bb0105","first-page":"1285","article-title":"The mucopolysaccharidoses: characterization by cranial MR imaging AJNR","volume":"14","author":"Lee","year":"1993","journal-title":"Am. J. Neuroradiol."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0110","doi-asserted-by":"crossref","first-page":"666","DOI":"10.1007\/s00234-004-1215-1","article-title":"Brain MRI findings in patients with mucopolysaccharidosis types I and II and mild clinical presentation","volume":"46","author":"Matheus","year":"2004","journal-title":"Neuroradiology"},{"key":"10.1016\/j.ymgme.2018.08.003_bb0115","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1016\/0730-725X(87)90120-2","article-title":"Magnetic resonance imaging in the diagnosis of the cranio-cervical manifestations of the mucopolysaccharidoses","volume":"5","author":"Kulkarni","year":"1987","journal-title":"Magn. Reson. Imaging"},{"key":"10.1016\/j.ymgme.2018.08.003_bb0120","doi-asserted-by":"crossref","first-page":"357","DOI":"10.1007\/s10545-012-9559-x","article-title":"Brain magnetic resonance imaging findings in patients with mucopolysaccharidosis VI","volume":"36","author":"Azevedo","year":"2013","journal-title":"J. Inherit. Metab. Dis."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0125","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1002\/ana.1098","article-title":"Brain magnetic resonance imaging in 23 patients with mucopolysaccharidoses and the effect of bone marrow transplantation","volume":"50","author":"Seto","year":"2001","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0130","doi-asserted-by":"crossref","first-page":"224","DOI":"10.1002\/ajmg.a.20515","article-title":"Correlation between cerebral MRI abnormalities and mental retardation in patients with mucopolysaccharidoses","volume":"125A","author":"Gabrielli","year":"2004","journal-title":"Am. J. Med. Genet. A"},{"key":"10.1016\/j.ymgme.2018.08.003_bb0135","doi-asserted-by":"crossref","first-page":"1319","DOI":"10.3174\/ajnr.A2032","article-title":"Correlation of automated volumetric analysis of brain MR imaging with cognitive impairment in a natural history study of mucopolysaccharidosis II AJNR","volume":"31","author":"Fan","year":"2010","journal-title":"Am. J. Neuroradiol."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0140","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1016\/j.ejpn.2013.11.014","article-title":"Mucopolysaccharidoses type I and II: new neuroimaging findings in the cerebellum","volume":"18","author":"Alqahtani","year":"2014","journal-title":"Eur. J. Paediatr. Neurol."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0145","doi-asserted-by":"crossref","first-page":"1158","DOI":"10.1097\/00005072-199710000-00010","article-title":"Human mucopolysaccharidosis IIID: clinical, biochemical, morphological and immunohistochemical characteristics","volume":"56","author":"Jones","year":"1997","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0150","doi-asserted-by":"crossref","first-page":"645","DOI":"10.1001\/archneur.1980.00500590069012","article-title":"Pathologic findings in mucopolysaccharidosis type IIIB (Sanfilippo's sydnrome B)","volume":"37","author":"Hadfield","year":"1980","journal-title":"Arch. Neurol."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0155","doi-asserted-by":"crossref","first-page":"189","DOI":"10.3109\/15513815.2010.547559","article-title":"Nodular lesions of choroid plexus in Hurler disease","volume":"30","author":"Lach","year":"2011","journal-title":"Fetal. Pediatr. Pathol."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0160","doi-asserted-by":"crossref","first-page":"198","DOI":"10.1016\/j.jpeds.2018.01.044","article-title":"Observational prospective natural history of patients with Sanfilippo syndrome type B","volume":"197","author":"Whitley","year":"2018","journal-title":"J. Pediatr."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0165","doi-asserted-by":"crossref","first-page":"547","DOI":"10.1007\/s00401-007-0325-3","article-title":"Mechanisms of neurodegeneration in mucopolysaccharidoses II and IIIB: analysis of human brain tissue","volume":"115","author":"Hamano","year":"2008","journal-title":"Acta Neuropathol."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0170","first-page":"115","article-title":"Morphological and biochemical studies of a case of mucopolysaccharidosis II (Hunter's syndrome)","volume":"26","author":"Nagashima","year":"1976","journal-title":"Acta Pathol. Jpn."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0175","first-page":"434","article-title":"Hurler's and Sanfilippo's variants of mucopolysaccharidosis. Cerebral pathology and lipid chemistry","volume":"91","author":"Dekaban","year":"1971","journal-title":"Arch. Pathol."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0180","doi-asserted-by":"crossref","first-page":"719","DOI":"10.1016\/S0009-9260(96)80246-7","article-title":"Magnetic resonance imaging of the brain, neck and cervical spine in mild Hunter's syndrome (mucopolysaccharidoses type II)","volume":"51","author":"Parsons","year":"1996","journal-title":"Clin. Radiol."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0185","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1016\/j.ymgme.2017.09.011","article-title":"Surgical management of neurological manifestations of mucopolysaccharidosis disorders","volume":"122S","author":"Alden","year":"2017","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0190","doi-asserted-by":"crossref","first-page":"455","DOI":"10.1007\/s10545-012-9574-y","article-title":"The CXCL12\/CXCR4\/CXCR7 ligand-receptor system regulates neuro-glio-vascular interactions and vessel growth during human brain development","volume":"36","author":"Virgintino","year":"2013","journal-title":"J. Inherit. Metab. Dis."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0195","doi-asserted-by":"crossref","first-page":"1566","DOI":"10.2174\/138161208784705504","article-title":"Lysosomal storage diseases and the blood-brain barrier","volume":"14","author":"Begley","year":"2008","journal-title":"Curr. Pharm. Des."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0200","doi-asserted-by":"crossref","first-page":"174","DOI":"10.1186\/1471-2377-13-174","article-title":"Blood-brain barrier impairment in MPS III patients","volume":"13","author":"Garbuzova-Davis","year":"2013","journal-title":"BMC Neurol."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0205","doi-asserted-by":"crossref","first-page":"1162","DOI":"10.1002\/ajmg.a.36424","article-title":"Mucopolysaccharidosis type IVA: evidence of primary and secondary central nervous system involvement","volume":"164A","author":"Borlot","year":"2014","journal-title":"Am. J. Med. Genet. A"},{"key":"10.1016\/j.ymgme.2018.08.003_bb0210","doi-asserted-by":"crossref","first-page":"57","DOI":"10.3109\/03008208609001973","article-title":"Ultrastructural and biochemical aspects of the Sanfilippo syndrome,\u2014type III genetic mucopolysaccharidosis","volume":"15","author":"Haust","year":"1986","journal-title":"Connect. Tissue Res."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0215","doi-asserted-by":"crossref","first-page":"611","DOI":"10.1007\/BF00686207","article-title":"Alterations in neuron morphology in mucopolysaccharidosis type I","volume":"75","author":"Walkley","year":"1988","journal-title":"A Golgi Study Acta Neuropathol"},{"key":"10.1016\/j.ymgme.2018.08.003_bb0220","doi-asserted-by":"crossref","first-page":"315","DOI":"10.1111\/j.1365-2990.1988.tb00891.x","article-title":"Focal dendritic swellings in Purkinje cells in mucopolysaccharidoses types I, II and III. A Golgi and ultrastructural study","volume":"14","author":"Ferrer","year":"1988","journal-title":"Neuropathol. Appl. Neurobiol."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0225","doi-asserted-by":"crossref","first-page":"1399","DOI":"10.1111\/j.1471-4159.1980.tb11220.x","article-title":"Mucopolysaccharidosis types IH, IS, II, and IIIA: glycosaminoglycans and lipids of isolated brain cells and other fractions from autopsied tissues","volume":"34","author":"Constantopoulos","year":"1980","journal-title":"J. Neurochem."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0230","doi-asserted-by":"crossref","first-page":"838","DOI":"10.1001\/archneur.1978.00500360062013","article-title":"Neuroanatomical and electroencephalographic correlations in Sanfilippo syndrome, type A","volume":"35","author":"Kriel","year":"1978","journal-title":"Arch. Neurol."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0235","doi-asserted-by":"crossref","first-page":"433","DOI":"10.1016\/j.semcdb.2004.03.002","article-title":"Secondary accumulation of gangliosides in lysosomal storage disorders","volume":"15","author":"Walkley","year":"2004","journal-title":"Semin. Cell Dev. Biol."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0240","doi-asserted-by":"crossref","DOI":"10.1038\/cddis.2013.430","article-title":"Murine neural stem cells model Hunter disease in vitro: glial cell-mediated neurodegeneration as a possible mechanism involved","volume":"4","author":"Fusar Poli","year":"2013","journal-title":"Cell Death Dis."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0245","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1016\/0304-4165(83)90225-8","article-title":"Decreased ganglioside neuraminidase activity in fibroblasts from mucopolysaccharidosis patients. Inhibition of the activity in vitro by sulfated glycosaminoglycans and other compounds","volume":"761","author":"Baumk\u00f6tter","year":"1983","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.ymgme.2018.08.003_bb0250","doi-asserted-by":"crossref","first-page":"2284","DOI":"10.1007\/s00330-005-2846-2","article-title":"Diffusion-weighted MR imaging in leukodystrophies","volume":"15","author":"Patay","year":"2005","journal-title":"Eur. Radiol."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0255","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1016\/j.nbd.2011.02.015","article-title":"Direct gene transfer to the CNS prevents emergence of neurologic disease in a murine model of mucopolysaccharidosis type I","volume":"43","author":"Wolf","year":"2011","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0260","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0172435","article-title":"Identification of age-dependent motor and neuropsychological behavioural abnormalities in a mouse model of Mucopolysaccharidosis type II","volume":"12","author":"Gleitz","year":"2017","journal-title":"PLoS One"},{"key":"10.1016\/j.ymgme.2018.08.003_bb0265","article-title":"Female mucopolysaccharidosis IIIA mice exhibit hyperactivity and a reduced sense of danger in the open field test","volume":"10","author":"Langford-Smith","year":"2011","journal-title":"PLoS One"},{"key":"10.1016\/j.ymgme.2018.08.003_bb0270","doi-asserted-by":"crossref","first-page":"336","DOI":"10.1093\/brain\/awu355","article-title":"Neuroinflammation, mitochondrial defects and neurodegeneration in mucopolysaccharidosis III type C mouse model","volume":"138","author":"Martins","year":"2015","journal-title":"Brain"},{"key":"10.1016\/j.ymgme.2018.08.003_bb0275","doi-asserted-by":"crossref","first-page":"673","DOI":"10.1111\/j.1601-183X.2011.00706.x","article-title":"Hyperactive behaviour in the mouse model of mucopolysaccharidosis IIIB in the open field and home cage environments","volume":"10","author":"Langford-Smith","year":"2011","journal-title":"Genes Brain Behav."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0280","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0035787","article-title":"Neuropathology in mouse models of mucopolysaccharidosis type I, IIIA and IIIB","volume":"7","author":"Wilkinson","year":"2012","journal-title":"PLoS One"},{"issue":"7","key":"10.1016\/j.ymgme.2018.08.003_bb0290","doi-asserted-by":"crossref","first-page":"2014","DOI":"10.1093\/brain\/awy126","article-title":"A novel adeno-associated virus capsid with enhanced neurotropism corrects a lysosomal transmembrane enzyme deficiency","volume":"141","author":"Tordo","year":"2018","journal-title":"Brain"},{"issue":"7","key":"10.1016\/j.ymgme.2018.08.003_bb0295","doi-asserted-by":"crossref","DOI":"10.15252\/emmm.201708730","article-title":"Brain targeted stem cell gene therapy corrects Mucopolysaccharidosis type II via multiple mechanisms","volume":"10","author":"Gleitz","year":"2018","journal-title":"Embo. Mol. Med."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0300","doi-asserted-by":"crossref","first-page":"56","DOI":"10.1111\/j.1651-2227.2007.00211.x","article-title":"Animal models for mucopolysaccharidosis disorders and their clinical relevance","volume":"96","author":"Haskins","year":"2007","journal-title":"Acta Paediatr."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0305","first-page":"27","volume":"112","author":"Haskins","year":"1983","journal-title":"Am. J. Pathol."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0310","doi-asserted-by":"crossref","first-page":"283","DOI":"10.1016\/j.ymgme.2014.10.008","article-title":"Development of cerebellar pathology in the canine model of mucopolysaccharidosis type IIIA (MPS IIIA)","volume":"113","author":"Hassiotis","year":"2014","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0315","doi-asserted-by":"crossref","first-page":"562","DOI":"10.1007\/s004010050562","article-title":"Neuropathology of murine mucopolysaccharidosis type VII","volume":"92","author":"Levy","year":"1996","journal-title":"Acta Neuropathol."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0320","doi-asserted-by":"crossref","first-page":"148","DOI":"10.1097\/00005072-199802000-00006","article-title":"Caprine mucopolysaccharidosis-IIID: clinical, biochemical, morphological and immunohistochemical characteristics","volume":"57","author":"Jones","year":"1998","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0325","doi-asserted-by":"crossref","first-page":"230","DOI":"10.1177\/030098589803500311","article-title":"Clinical and morphologic features of mucopolysaccharidosis type II in a dog: naturally occurring model of hunter syndrome","volume":"35","author":"Wilkerson","year":"1998","journal-title":"Vet. Pathol."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0330","doi-asserted-by":"crossref","first-page":"569","DOI":"10.1354\/vp.44-5-569","article-title":"Pathology of mucopolysaccharidosis IIIA in huntaway dogs","volume":"44","author":"Jolly","year":"2007","journal-title":"Vet. Pathol."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0335","doi-asserted-by":"crossref","first-page":"160","DOI":"10.1177\/0300985814529314","article-title":"Pathological and biochemical studies of mucopolysaccharidosis type IIIB (Sanfilippo syndrome type B) in juvenile emus (Dromaius novaehollandiae)","volume":"52","author":"Palmieri","year":"2015","journal-title":"Vet. Pathol."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0340","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1111\/j.1399-0004.1998.tb02745.x","article-title":"Murine MPS I: insights into the pathogenesis of Hurler syndrome","volume":"53","author":"Russell","year":"1998","journal-title":"Clin. Genet."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0345","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."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0350","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0002296","article-title":"Early neurodegeneration progresses independently of microglial activation by heparan sulfate in the brain of mucopolysaccharidosis IIIB mice","volume":"3","author":"Ausseil","year":"2008","journal-title":"PLoS One"},{"key":"10.1016\/j.ymgme.2018.08.003_bb0355","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1002\/jnr.10278","article-title":"Attenuated plasticity in neurons and astrocytes in the mouse model of Sanfilippo syndrome type B","volume":"69","author":"Li","year":"2002","journal-title":"J. Neurosci. Res."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0360","doi-asserted-by":"crossref","first-page":"1030","DOI":"10.1038\/nature05817","article-title":"Heparan sulphate proteoglycans fine-tune mammalian physiology","volume":"446","author":"Bishop","year":"2007","journal-title":"Nature"},{"key":"10.1016\/j.ymgme.2018.08.003_bb0365","doi-asserted-by":"crossref","first-page":"36194","DOI":"10.1074\/jbc.M114.599944","article-title":"Heparan sulfate inhibits hematopoietic stem and progenitor cell migration and engraftment in Mucopolysaccharidosis I","volume":"289","author":"Watson","year":"2014","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0370","doi-asserted-by":"crossref","first-page":"37515","DOI":"10.1074\/jbc.M111.287474","article-title":"Mucopolysaccharidosis type I, unique structure of accumulated heparan sulfate and increased N-sulfotransferase activity in mice lacking \u03b1-l-iduronidase","volume":"286","author":"Holley","year":"2011","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0375","doi-asserted-by":"crossref","first-page":"775","DOI":"10.1016\/j.jmb.2014.09.012","article-title":"Heparan sulfate saccharides modify focal adhesions: implication in mucopolysaccharidosis neuropathophysiology","volume":"427","author":"Bruy\u00e8re","year":"2015","journal-title":"J. Mol. Biol."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0380","first-page":"1088","article-title":"Distribution of heparan sulfate oligosaccharides in murine mucopolysaccharidosis type IIIA","volume":"4","author":"Mason","year":"2014","journal-title":"Meta"},{"key":"10.1016\/j.ymgme.2018.08.003_bb0385","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1007\/s11011-010-9230-x","article-title":"Differential distribution of heparan sulfate glycoforms and elevated expression of heparan sulfate biosynthetic enzyme genes in the brain of mucopolysaccharidosis IIIB mice","volume":"26","author":"McCarty","year":"2011","journal-title":"Metab. Brain Dis."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0390","doi-asserted-by":"crossref","first-page":"271","DOI":"10.1007\/s10545-012-9535-5","article-title":"Heparan sulfate derived disaccharides in plasma and total urinary excretion of glycosaminoglycans correlate with disease severity in Sanfilippo disease","volume":"36","author":"Ruijter De","year":"2013","journal-title":"J. Inherit. Metab. Dis."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0395","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.2018.08.003_bb0400","doi-asserted-by":"crossref","first-page":"22936","DOI":"10.1074\/jbc.273.36.22936","article-title":"Heparan sulfate oligosaccharides require 6-O-sulfation for promotion of basic fibroblast growth factor mitogenic activity","volume":"273","author":"Pye","year":"1998","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0405","doi-asserted-by":"crossref","first-page":"438","DOI":"10.1042\/BST0340438","article-title":"Multiprotein signalling complexes: regional assembly on heparan sulphate","volume":"34","author":"Gallagher","year":"2006","journal-title":"Biochem. Soc. Trans."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0410","series-title":"Functional Abnormalities of Heparan Sulfate in Mucopolysaccharidosis-I Are Associated with Defective Biologic Activity of FGF-2 on Human Multipotent Progenitor Cells Blood 106 (2005)","author":"Pan","year":"1956"},{"key":"10.1016\/j.ymgme.2018.08.003_bb0415","doi-asserted-by":"crossref","first-page":"760","DOI":"10.1097\/nen.0b013e31812571c8","article-title":"Abnormalities in neural progenitor cells in a dog model of lysosomal storage disease","volume":"66","author":"Walton","year":"2007","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0420","first-page":"224","article-title":"Two-dimensional NMR spectroscopy of urinary glycosaminoglycans from patients with different mucopolysaccharidoses NMR","volume":"16","author":"Hochuli","year":"2003","journal-title":"Biomedicine"},{"key":"10.1016\/j.ymgme.2018.08.003_bb0425","doi-asserted-by":"crossref","first-page":"612","DOI":"10.1002\/jnr.21134","article-title":"Cytokines, neurotrophins, and oxidative stress in brain disease from mucopolysaccharidosis IIIB","volume":"85","author":"Villani","year":"2007","journal-title":"J. Neurosci. Res."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0430","doi-asserted-by":"crossref","first-page":"424","DOI":"10.1002\/jnr.23497","article-title":"Oxidative stress is independent of inflammation in the neurodegenerative Sanfilippo syndrome type B","volume":"93","author":"Trudel","year":"2015","journal-title":"J. Neurosci. Res."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0435","doi-asserted-by":"crossref","first-page":"18","DOI":"10.1016\/j.ymgme.2011.01.015","article-title":"Neuroinflammatory and oxidative stress phenomena in MPS IIIA mouse model: the positive effect of long-term aspirin treatment","volume":"103","author":"Arfi","year":"2011","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0440","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1007\/s10545-013-9613-3","article-title":"Mucopolysaccharide diseases: a complex interplay between neuroinflammation, microglial activation and adaptive immunity","volume":"37","author":"Archer","year":"2014","journal-title":"J. Inherit. Metab. Dis."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0445","doi-asserted-by":"crossref","first-page":"283","DOI":"10.1038\/nrm3565","article-title":"Signals from the lysosome: a control centre for cellular clearance and energy metabolism","volume":"14","author":"Settembre","year":"2013","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0450","first-page":"531","article-title":"Lysosomal labilization IUBMB","volume":"58","author":"Terman","year":"2006","journal-title":"Lifestyles"},{"key":"10.1016\/j.ymgme.2018.08.003_bb0455","article-title":"Crosstalk between 2 organelles: Lysosomal storage of heparan sulfate causes mitochondrial defects and neuronal death in mucopolysaccharidosis III type C","volume":"3","author":"Pshezhetsky","year":"2015","journal-title":"Rare Dis."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0460","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0027461","article-title":"Defects in the medial entorhinal cortex and dentate gyrus in the mouse model of Sanfilippo syndrome type B","volume":"6","author":"Ohmi","year":"2011","journal-title":"PLoS One"},{"key":"10.1016\/j.ymgme.2018.08.003_bb0465","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1016\/j.brainres.2009.03.071","article-title":"Mucopolysaccharidosis IIIB: oxidative damage and cytotoxic cell involvement in the neuronal pathogenesis","volume":"1279","author":"Villani","year":"2009","journal-title":"Brain Res."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0470","doi-asserted-by":"crossref","first-page":"443","DOI":"10.1007\/s10571-008-9335-5","article-title":"Alterations in oxidative markers in the cerebellum and peripheral organs in MPS I mice","volume":"29","author":"Reolon","year":"2009","journal-title":"Cell. Mol. Neurobiol."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0475","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1016\/j.cca.2007.09.008","article-title":"Mutational and oxidative stress analysis in patients with mucopolysaccharidosis type I undergoing enzyme replacement therapy","volume":"387","author":"Pereira","year":"2008","journal-title":"Clin. Chim. Acta"},{"key":"10.1016\/j.ymgme.2018.08.003_bb0480","doi-asserted-by":"crossref","first-page":"361","DOI":"10.1111\/j.1474-9726.2007.00294.x","article-title":"Oxidative stress and aberrant signaling in aging and cognitive decline","volume":"6","author":"Droge","year":"2007","journal-title":"Aging Cell"},{"key":"10.1016\/j.ymgme.2018.08.003_bb0485","doi-asserted-by":"crossref","first-page":"297","DOI":"10.4161\/oxim.2.5.9541","article-title":"Tumor necrosis factor-\u03b1 and apoptosis signal-regulating kinase 1 control reactive oxygen species release, mitochondrial autophagy, and c-Jun N-terminal kinase\/p38 phosphorylation during necrotizing enterocolitis","volume":"2","author":"Baregamian","year":"2009","journal-title":"Oxidative Med. Cell. Longev."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0490","doi-asserted-by":"crossref","first-page":"1724","DOI":"10.1093\/brain\/aws095","article-title":"Contribution of brain inflammation to neuronal cell death in neuronopathic forms of Gaucher's disease","volume":"135","author":"Vitner","year":"2012","journal-title":"Brain"},{"key":"10.1016\/j.ymgme.2018.08.003_bb0495","doi-asserted-by":"crossref","first-page":"726","DOI":"10.1016\/j.bbamcr.2008.11.014","article-title":"Secondary lipid accumulation in lysosomal disease","volume":"1793","author":"Walkley","year":"2009","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.ymgme.2018.08.003_bb0500","doi-asserted-by":"crossref","first-page":"415","DOI":"10.1002\/cne.20355","article-title":"Differential subcellular localization of cholesterol, gangliosides, and glycosaminoglycans in murine models of mucopolysaccharide storage disorders","volume":"480","author":"McGlynn","year":"2004","journal-title":"J. Comp. Neurol."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0505","doi-asserted-by":"crossref","first-page":"1028","DOI":"10.1093\/cercor\/10.10.1028","article-title":"Gangliosides as modulators of dendritogenesis in normal and storage disease-affected pyramidal neurons","volume":"10","author":"Walkley","year":"2000","journal-title":"Cereb. Cortex"},{"key":"10.1016\/j.ymgme.2018.08.003_bb0510","series-title":"Apoptotic Cell Death in Mouse Models of GM2 Gangliosidosis and Observations on Human Tay-Sachs and Sandhoff Diseases Hum Mol Genet 6 (1997)","author":"Huang","year":"1879"},{"key":"10.1016\/j.ymgme.2018.08.003_bb0515","doi-asserted-by":"crossref","first-page":"8332","DOI":"10.1073\/pnas.0903223106","article-title":"Sanfilippo syndrome type B, a lysosomal storage disease, is also a tauopathy","volume":"106","author":"Ohmi","year":"2009","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0520","doi-asserted-by":"crossref","first-page":"393","DOI":"10.1016\/j.ymgme.2006.11.006","article-title":"Lysosomal accumulation of SCMAS (subunit c of mitochondrial ATP synthase) in neurons of the mouse model of mucopolysaccharidosis III B","volume":"90","author":"Ryazantsev","year":"2007","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0525","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0000772","article-title":"Development of sensory, motor and behavioral deficits in the murine model of Sanfilippo syndrome type B","volume":"2","author":"Heldermon","year":"2007","journal-title":"PLoS One"},{"key":"10.1016\/j.ymgme.2018.08.003_bb0530","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1016\/j.mcn.2009.01.001","article-title":"Enhanced degradation of synaptophysin by the proteasome in mucopolysaccharidosis type IIIB","volume":"41","author":"Vitry","year":"2009","journal-title":"Mol. Cell. Neurosci."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0535","doi-asserted-by":"crossref","first-page":"S39","DOI":"10.1016\/j.ymgme.2015.12.234","article-title":"Synaptic dysfunction in Sanfilippo syndrome type C","volume":"117","author":"Aragao de","year":"2016","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0540","doi-asserted-by":"crossref","first-page":"331","DOI":"10.1111\/ped.12636","article-title":"Sanfilippo syndrome: overall review","volume":"57","author":"Andrade","year":"2015","journal-title":"Pediatr. Int."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0545","first-page":"655","article-title":"Establishment of glycosaminoglycan assays for mucopolysaccharidoses","volume":"4","author":"Tomatsu","year":"2014","journal-title":"Meta"},{"key":"10.1016\/j.ymgme.2018.08.003_bb0550","article-title":"Fast, sensitive method for trisaccharide biomarker detection in mucopolysaccharidosis type 1","volume":"8","author":"Makino","year":"2018","journal-title":"Sci. Rep."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0555","doi-asserted-by":"crossref","first-page":"1320","DOI":"10.3324\/haematol.2011.058644","article-title":"Hematopoietic stem cell transplantation improves the high incidence of neutralizing allo-antibodies observed in Hurler's syndrome after pharmacological enzyme replacement therapy","volume":"97","author":"Saif","year":"2012","journal-title":"Haematologica"},{"key":"10.1016\/j.ymgme.2018.08.003_bb0560","doi-asserted-by":"crossref","first-page":"609","DOI":"10.1016\/j.jpeds.2008.11.005","article-title":"Improved metabolic correction in patients with lysosomal storage disease treated with hematopoietic stem cell transplant compared with enzyme replacement therapy","volume":"154","author":"Wynn","year":"2009","journal-title":"J. Pediatr."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0565","doi-asserted-by":"crossref","first-page":"499","DOI":"10.1007\/s10545-010-9254-8","article-title":"Heparin cofactor II-thrombin complex and dermatan sulphate:chondroitin sulphate ratio are biomarkers of short- and long-term treatment effects in mucopolysaccharide diseases","volume":"34","author":"Langford-Smith","year":"2011","journal-title":"J. Inherit. Metab. Dis."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0570","doi-asserted-by":"crossref","first-page":"129","DOI":"10.1016\/j.ymgme.2014.10.012","article-title":"Biomarker responses correlate with antibody status in mucopolysaccharidosis type I patients on long-term enzyme replacement therapy","volume":"114","author":"Langereis","year":"2015","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0575","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.2018.08.003_bb0580","doi-asserted-by":"crossref","first-page":"456","DOI":"10.1016\/j.ymgme.2008.05.001","article-title":"Heparin cofactor II-thrombin complex: a biomarker of MPS disease","volume":"94","author":"Randall","year":"2008","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0585","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1016\/j.ymgme.2009.10.175","article-title":"Evaluation of heparin cofactor II-thrombin complex as a biomarker on blood spots from mucopolysaccharidosis I, IIIA and IIIB mice","volume":"99","author":"Langford-Smith","year":"2010","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0590","doi-asserted-by":"crossref","first-page":"222","DOI":"10.1073\/pnas.0912937107","article-title":"Involvement of the toll-like receptor 4 pathway and use of TNF-a antagonists for treatment of the mucopolysaccharidoses","volume":"107","author":"Simonaro","year":"2010","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0595","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1007\/s10545-010-9051-4","article-title":"Serum MIP-1 \u03b1 level: a biomarker for the follow-up of lentiviral therapy in mucopolysaccharidosis IIIB mice","volume":"33","author":"Di Natale","year":"2010","journal-title":"J. Inherit. Metab. Dis."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0600","doi-asserted-by":"crossref","first-page":"736","DOI":"10.1046\/j.1471-4159.2002.01041.x","article-title":"Inositol and higher inositol phosphates in neural tissues: homeostasis, metabolism and functional significance","volume":"82","author":"Fisher","year":"2002","journal-title":"J. Neurochem."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0605","doi-asserted-by":"crossref","first-page":"897","DOI":"10.1212\/01.WNL.0000137420.58346.9F","article-title":"Elevation of myoinositol is associated with disease containment in progressive multifocal leukoencephalopathy","volume":"63","author":"Katz-Brull","year":"2004","journal-title":"Neurology"},{"key":"10.1016\/j.ymgme.2018.08.003_bb0610","doi-asserted-by":"crossref","first-page":"256","DOI":"10.1002\/mrm.20366","article-title":"MR spectroscopic evidence for glial increase but not for neuro-axonal damage in MS normal-appearing white matter","volume":"53","author":"Vrenken","year":"2005","journal-title":"Magn. Reson. Med."},{"key":"10.1016\/j.ymgme.2018.08.003_bb0615","first-page":"752","article-title":"Relationships between astrogliosis and 1H MR spectroscopic measures of brain choline\/creatine and myo-inositol\/creatine in a primate model AJNR","volume":"26","author":"Kim","year":"2005","journal-title":"Am. J. Neuroradiol."}],"container-title":["Molecular Genetics and Metabolism"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1096719218303068?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1096719218303068?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2024,6,2]],"date-time":"2024-06-02T03:19:29Z","timestamp":1717298369000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1096719218303068"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,12]]},"references-count":122,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2018,12]]}},"alternative-id":["S1096719218303068"],"URL":"https:\/\/doi.org\/10.1016\/j.ymgme.2018.08.003","relation":{},"ISSN":["1096-7192"],"issn-type":[{"value":"1096-7192","type":"print"}],"subject":[],"published":{"date-parts":[[2018,12]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Anatomical changes and pathophysiology of the brain in mucopolysaccharidosis disorders","name":"articletitle","label":"Article Title"},{"value":"Molecular Genetics and Metabolism","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.ymgme.2018.08.003","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2018 The Authors. Published by Elsevier Inc.","name":"copyright","label":"Copyright"}]}}