{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T13:37:38Z","timestamp":1790861858511,"version":"4.1.0"},"reference-count":37,"publisher":"Public Library of Science (PLoS)","issue":"12","license":[{"start":{"date-parts":[[2010,12,1]],"date-time":"2010-12-01T00:00:00Z","timestamp":1291161600000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["www.plosone.org"],"crossmark-restriction":false},"short-container-title":["PLoS ONE"],"DOI":"10.1371\/journal.pone.0014192","type":"journal-article","created":{"date-parts":[[2010,12,1]],"date-time":"2010-12-01T20:48:20Z","timestamp":1291236500000},"page":"e14192","update-policy":"https:\/\/doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":127,"title":["Genistein Improves Neuropathology and Corrects Behaviour in a Mouse Model of Neurodegenerative Metabolic Disease"],"prefix":"10.1371","volume":"5","author":[{"given":"Marcelina","family":"Malinowska","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fiona L.","family":"Wilkinson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kia J.","family":"Langford-Smith","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alex","family":"Langford-Smith","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jillian R.","family":"Brown","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brett E.","family":"Crawford","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie T.","family":"Vanier","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Grzegorz","family":"Grynkiewicz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rob F.","family":"Wynn","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. Ed","family":"Wraith","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Grzegorz","family":"Wegrzyn","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brian W.","family":"Bigger","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"340","published-online":{"date-parts":[[2010,12,1]]},"reference":[{"key":"ref1","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1007\/s004399900075","article-title":"The frequency of lysosomal storage diseases in The Netherlands.","volume":"105","author":"BJ Poorthuis","year":"1999","journal-title":"Hum Genet"},{"key":"ref2","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1053\/siny.2001.0088","article-title":"Lysosomal disorders.","volume":"7","author":"JE Wraith","year":"2002","journal-title":"Semin Neonatol"},{"key":"ref3","doi-asserted-by":"crossref","first-page":"442","DOI":"10.1007\/s10545-006-0239-6","article-title":"Limitations of enzyme replacement therapy: current and future.","volume":"29","author":"JE Wraith","year":"2006","journal-title":"J Inherit Metab Dis"},{"key":"ref4","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":"RF Wynn","year":"2009","journal-title":"J Pediatr"},{"key":"ref5","first-page":"88","article-title":"Substrate reduction therapy.","volume":"Paediatr","author":"FM Platt","year":"2008","journal-title":"Acta"},{"key":"ref6","doi-asserted-by":"crossref","first-page":"5592","DOI":"10.1016\/S0021-9258(18)45614-1","article-title":"Genistein, a specific inhibitor of tyrosine-specific protein kinases.","volume":"262","author":"T Akiyama","year":"1987","journal-title":"J Biol Chem"},{"key":"ref7","doi-asserted-by":"crossref","first-page":"846","DOI":"10.1038\/sj.ejhg.5201623","article-title":"Genistein-mediated inhibition of glycosaminoglycan synthesis as a basis for gene expression-targeted isoflavone therapy for mucopolysaccharidoses.","volume":"14","author":"E Piotrowska","year":"2006","journal-title":"Eur J Hum Genet"},{"key":"ref8","doi-asserted-by":"crossref","first-page":"1461","DOI":"10.1016\/j.fct.2005.02.017","article-title":"Subchronic and chronic safety studies with genistein in dogs.","volume":"43","author":"RM McClain","year":"2005","journal-title":"Food Chem Toxicol"},{"key":"ref9","doi-asserted-by":"crossref","first-page":"56","DOI":"10.1016\/j.fct.2005.05.021","article-title":"Acute, subchronic and chronic safety studies with genistein in rats.","volume":"44","author":"R Michael McClain","year":"2006","journal-title":"Food Chem Toxicol"},{"key":"ref10","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1016\/j.chroma.2004.10.048","article-title":"Concurrent measurement of unbound genistein in the blood, brain and bile of anesthetized rats using microdialysis and its pharmacokinetic application.","volume":"1073","author":"TH Tsai","year":"2005","journal-title":"J Chromatogr A"},{"key":"ref11","article-title":"Sanfilippo syndrome: A mini-review.","author":"MJ Valstar","year":"2008","journal-title":"J Inherit Metab Dis"},{"key":"ref12","doi-asserted-by":"crossref","first-page":"166","DOI":"10.1016\/j.curtheres.2008.04.002","article-title":"Genistin-Rich Soy Isoflavone Extract in Substrate Reduction Therapy for Sanfilippo Syndrome: An Open-Label, Pilot Study in 10 Pediatric Patients.","volume":"69","author":"E Piotrowska","year":"2008","journal-title":"Curr Ther Res"},{"key":"ref13","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1016\/j.ymgme.2009.06.013","article-title":"Genistein reduces lysosomal storage in peripheral tissues of mucopolysaccharide IIIB mice.","volume":"98","author":"M Malinowska","year":"2009","journal-title":"Mol Genet Metab"},{"key":"ref14","doi-asserted-by":"crossref","first-page":"491","DOI":"10.1016\/S0197-0186(96)00086-1","article-title":"Protein tyrosine kinase inhibitors decrease lipopolysaccharide-induced proinflammatory cytokine production in mixed glia, microglia-enriched or astrocyte-enriched cultures.","volume":"30","author":"LY Kong","year":"1997","journal-title":"Neurochem Int"},{"key":"ref15","doi-asserted-by":"crossref","first-page":"267","DOI":"10.1097\/00001756-200502280-00013","article-title":"Genistein protects dopaminergic neurons by inhibiting microglial activation.","volume":"16","author":"X Wang","year":"2005","journal-title":"Neuroreport"},{"key":"ref16","doi-asserted-by":"crossref","first-page":"212","DOI":"10.1016\/j.bbr.2010.01.045","article-title":"Circadian rhythm and suprachiasmatic nucleus alterations in the mouse model of mucopolysaccharidosis IIIB.","volume":"209","author":"MM Canal","year":"2010","journal-title":"Behav Brain Res"},{"key":"ref17","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":"K Ohmi","year":"2003","journal-title":"Proc Natl Acad Sci U S A"},{"key":"ref18","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1002\/jnr.22211","article-title":"Immune activation or immunomodulation in the brains of MPS IIIB mice? Commentary on \u201cInnate and adaptive immune activation in the brain of MPS IIIB mouse model\u201d.","volume":"88","author":"LD Archer","journal-title":"J Neurosci Res"},{"key":"ref19","doi-asserted-by":"crossref","first-page":"978","DOI":"10.1002\/jnr.21912","article-title":"Innate and adaptive immune activation in the brain of MPS IIIB mouse model.","volume":"87","author":"J DiRosario","year":"2009","journal-title":"J Neurosci Res"},{"key":"ref20","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":"R McGlynn","year":"2004","journal-title":"J Comp Neurol"},{"key":"ref21","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":"SU Walkley","year":"2009","journal-title":"Biochim Biophys Acta"},{"key":"ref22","doi-asserted-by":"crossref","first-page":"1117","DOI":"10.1126\/science.1064335","article-title":"SNARE function analyzed in synaptobrevin\/VAMP knockout mice.","volume":"294","author":"S Schoch","year":"2001","journal-title":"Science"},{"key":"ref23","doi-asserted-by":"crossref","first-page":"10229","DOI":"10.1523\/JNEUROSCI.3558-04.2004","article-title":"Improved behavior and neuropathology in the mouse model of Sanfilippo type IIIB disease after adeno-associated virus-mediated gene transfer in the striatum.","volume":"24","author":"A Cressant","year":"2004","journal-title":"J Neurosci"},{"key":"ref24","doi-asserted-by":"crossref","first-page":"6388","DOI":"10.1073\/pnas.96.11.6388","article-title":"Delayed symptom onset and increased life expectancy in Sandhoff disease mice treated with N-butyldeoxynojirimycin.","volume":"96","author":"M Jeyakumar","year":"1999","journal-title":"Proc Natl Acad Sci U S A"},{"key":"ref25","doi-asserted-by":"crossref","first-page":"e2296","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":"J Ausseil","year":"2008","journal-title":"PLoS ONE"},{"key":"ref26","doi-asserted-by":"crossref","first-page":"983","DOI":"10.1016\/S0197-4580(01)00299-8","article-title":"Ibuprofen effects on Alzheimer pathology and open field activity in APPsw transgenic mice.","volume":"22","author":"GP Lim","year":"2001","journal-title":"Neurobiol Aging"},{"key":"ref27","doi-asserted-by":"crossref","first-page":"201","DOI":"10.1016\/S0197-4580(98)00047-5","article-title":"Indomethacin reverses the microglial response to amyloid beta-protein.","volume":"19","author":"EE Netland","year":"1998","journal-title":"Neurobiol Aging"},{"key":"ref28","doi-asserted-by":"crossref","first-page":"642","DOI":"10.1002\/ana.20242","article-title":"NSAIDs increase survival in the Sandhoff disease mouse: synergy with N-butyldeoxynojirimycin.","volume":"56","author":"M Jeyakumar","year":"2004","journal-title":"Ann Neurol"},{"key":"ref29","article-title":"What&apos;s wrong with my mouse?","author":"JN Crawley","year":"2007"},{"key":"ref30","first-page":"139","article-title":"Novel pathologic findings associated with urinary retention in a mouse model of mucopolysaccharidosis type IIIB.","volume":"59","author":"SI Gografe","year":"2009","journal-title":"Comp Med"},{"key":"ref31","doi-asserted-by":"crossref","first-page":"2307","DOI":"10.1093\/jn\/125.9.2307","article-title":"Bioavailability of soybean isoflavones depends upon gut microflora in women.","volume":"125","author":"X Xu","year":"1995","journal-title":"J Nutr"},{"key":"ref32","doi-asserted-by":"crossref","first-page":"1485","DOI":"10.1038\/sj.bjc.6604321","article-title":"The role of early life genistein exposures in modifying breast cancer risk.","volume":"98","author":"A Warri","year":"2008","journal-title":"Br J Cancer"},{"key":"ref33","doi-asserted-by":"crossref","first-page":"839","DOI":"10.7326\/0003-4819-146-12-200706190-00005","article-title":"Effects of the phytoestrogen genistein on bone metabolism in osteopenic postmenopausal women: a randomized trial.","volume":"146","author":"H Marini","year":"2007","journal-title":"Ann Intern Med"},{"key":"ref34","doi-asserted-by":"crossref","first-page":"1260","DOI":"10.1093\/jn\/127.7.1260","article-title":"Intestinal uptake and biliary excretion of the isoflavone genistein in rats.","volume":"127","author":"J Sfakianos","year":"1997","journal-title":"J Nutr"},{"key":"ref35","doi-asserted-by":"crossref","first-page":"14505","DOI":"10.1073\/pnas.96.25.14505","article-title":"Mouse model of Sanfilippo syndrome type B produced by targeted disruption of the gene encoding alpha-N-acetylglucosaminidase.","volume":"96","author":"HH Li","year":"1999","journal-title":"Proc Natl Acad Sci U S A"},{"key":"ref36","article-title":"The Mouse Brain in Stereotaxic Coordinates.","author":"F Paxinos","year":"2004"},{"key":"ref37","doi-asserted-by":"crossref","first-page":"483","DOI":"10.1093\/glycob\/cwn028","article-title":"A simplified and sensitive fluorescent method for disaccharide analysis of both heparan sulfate and chondroitin\/dermatan sulfates from biological samples.","volume":"18","author":"JA Deakin","year":"2008","journal-title":"Glycobiology"}],"container-title":["PLoS ONE"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/dx.plos.org\/10.1371\/journal.pone.0014192","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,11,14]],"date-time":"2021-11-14T13:41:56Z","timestamp":1636897316000},"score":1,"resource":{"primary":{"URL":"https:\/\/dx.plos.org\/10.1371\/journal.pone.0014192"}},"subtitle":[],"editor":[{"given":"Maria A.","family":"Deli","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2010,12,1]]},"references-count":37,"journal-issue":{"issue":"12","published-online":{"date-parts":[[2010,12,1]]}},"URL":"https:\/\/doi.org\/10.1371\/journal.pone.0014192","relation":{},"ISSN":["1932-6203"],"issn-type":[{"value":"1932-6203","type":"electronic"}],"subject":[],"published":{"date-parts":[[2010,12,1]]}}}