{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T21:03:40Z","timestamp":1791147820369,"version":"4.1.0"},"reference-count":35,"publisher":"National Academy of Sciences","issue":"20","content-domain":{"domain":["www.pnas.org"],"crossmark-restriction":true},"short-container-title":["Proc. Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[2009,5,19]]},"abstract":"<jats:p>\n                    Sanfilippo syndrome type B (mucopolysaccharidosis III B, MPS III B) is an autosomal recessive, neurodegenerative disease of children, characterized by profound mental retardation and dementia. The primary cause is mutation in the\n                    <jats:italic>NAGLU<\/jats:italic>\n                    gene, resulting in deficiency of \u03b1-\n                    <jats:italic>N<\/jats:italic>\n                    -acetylglucosaminidase and lysosomal accumulation of heparan sulfate. In the mouse model of MPS III B, neurons and microglia display the characteristic vacuolation of lysosomal storage of undegraded substrate, but neurons in the medial entorhinal cortex (MEC) display accumulation of several additional substances. We used whole genome microarray analysis to examine differential gene expression in MEC neurons isolated by laser capture microdissection from\n                    <jats:italic>Naglu<\/jats:italic>\n                    <jats:sup>\u2212\/\u2212<\/jats:sup>\n                    and\n                    <jats:italic>Naglu<\/jats:italic>\n                    <jats:sup>+\/\u2212<\/jats:sup>\n                    mice. Neurons from the lateral entorhinal cortex (LEC) were used as tissue controls. The highest increase in gene expression (6- to 7-fold between mutant and control) in MEC and LEC neurons was that of\n                    <jats:italic>Lyzs<\/jats:italic>\n                    , which encodes lysozyme, but accumulation of lysozyme protein was seen in MEC neurons only. Because of a report that lysozyme induced the formation of hyperphosphorylated tau (P-tau) in cultured neurons, we searched for P-tau by immunohistochemistry. P-tau was found in MEC of\n                    <jats:italic>Naglu<\/jats:italic>\n                    <jats:sup>\u2212\/\u2212<\/jats:sup>\n                    mice, in the same neurons as lysozyme. In older mutant mice, it was also seen in the dentate gyrus, an area important for memory. Electron microscopy of dentate gyrus neurons showed cytoplasmic inclusions of paired helical filaments, P-tau aggregates characteristic of tauopathies\u2014a group of age-related dementias that include Alzheimer disease. Our findings indicate that the Sanfilippo syndrome type B should also be considered a tauopathy.\n                  <\/jats:p>","DOI":"10.1073\/pnas.0903223106","type":"journal-article","created":{"date-parts":[[2009,5,5]],"date-time":"2009-05-05T22:44:19Z","timestamp":1241563459000},"page":"8332-8337","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":129,"title":["Sanfilippo syndrome type B, a lysosomal storage disease, is also a tauopathy"],"prefix":"10.1073","volume":"106","author":[{"given":"Kazuhiro","family":"Ohmi","sequence":"first","affiliation":[{"name":"Departments of aBiological Chemistry,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lili C.","family":"Kudo","sequence":"additional","affiliation":[{"name":"Neurology and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sergey","family":"Ryazantsev","sequence":"additional","affiliation":[{"name":"Departments of aBiological Chemistry,"},{"name":"Electron Imaging Center for Nanomachines at California NanoSystems Institute, University of California at Los Angeles, Los Angeles, CA 90095;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hui-Zhi","family":"Zhao","sequence":"additional","affiliation":[{"name":"Departments of aBiological Chemistry,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stanislav L.","family":"Karsten","sequence":"additional","affiliation":[{"name":"Neurology and"},{"name":"Obstetrics and Gynecology, David Geffen School of Medicine at University of California at Los Angeles, Los Angeles, CA 90095;"},{"name":"Division of Neuroscience, Los Angeles Biomedical Research Institute at Harbor-University of California at Los Angeles Medical Center, Torrance, CA 90502"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth F.","family":"Neufeld","sequence":"additional","affiliation":[{"name":"Departments of aBiological Chemistry,"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"341","published-online":{"date-parts":[[2009,5,19]]},"reference":[{"key":"e_1_3_3_1_2","first-page":"3421","volume-title":"The Metabolic and Molecular Bases of Inherited Disease","author":"Neufeld EF","year":"2001","unstructured":"EF Neufeld, J Muenzer, The mucopolysaccharidoses. 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