{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,3]],"date-time":"2026-05-03T10:04:45Z","timestamp":1777802685790,"version":"3.51.4"},"reference-count":38,"publisher":"SAGE Publications","issue":"9","license":[{"start":{"date-parts":[[2013,9,1]],"date-time":"2013-09-01T00:00:00Z","timestamp":1377993600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["J Child Neurol"],"published-print":{"date-parts":[[2013,9]]},"abstract":"<jats:p>\n                    The neuronal ceroid lipofuscinoses, a family of neurodegenerative lysosomal storage disorders, represent the most common cause of pediatric-onset neurodegeneration. The infantile form has a devastatingly early onset and one of the fastest-progressing disease courses. Despite decades of research, the molecular mechanisms driving neuronal loss in infantile neuronal ceroid lipofuscinosis remain unknown. We have previously shown that N-methyl-d-aspartate (NMDA)\u2013type glutamate receptors in the Ppt1\n                    <jats:sup>\u2013\/\u2013<\/jats:sup>\n                    mouse model of this disease exhibit a hyperfunctional phenotype and postulate that aberrant glutamatergic activity may contribute to neural pathology in both the mouse model and human patients. To test this hypothesis, we treated Ppt1\n                    <jats:sup>\u2013\/\u2013<\/jats:sup>\n                    mice with the NMDA receptor antagonist memantine and assessed their response to the drug using an accelerating rotarod. At 20 mg\/kg, memantine treatment induced a delayed but notable improvement in Ppt1\n                    <jats:sup>\u2013\/\u2013<\/jats:sup>\n                    mice. Much remains to be assessed before moving to patient trials, but these results suggest memantine has potential as a treatment.\n                  <\/jats:p>","DOI":"10.1177\/0883073813494480","type":"journal-article","created":{"date-parts":[[2013,9,6]],"date-time":"2013-09-06T08:39:19Z","timestamp":1378456759000},"page":"1159-1168","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":14,"title":["Treatment of the\n                    <i>\n                      Ppt1\n                      <sup>\u2013\/\u2013<\/sup>\n                    <\/i>\n                    Mouse Model of Infantile Neuronal Ceroid Lipofuscinosis With the\n                    <i>N<\/i>\n                    -methyl-\n                    <scp>d<\/scp>\n                    -aspartate (NMDA) Receptor Antagonist Memantine"],"prefix":"10.1177","volume":"28","author":[{"given":"Rozzy","family":"Finn","sequence":"first","affiliation":[{"name":"Sanford Children\u2019s Health Research Center, Sanford Research\/USD, Sioux Falls, SD, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Attila D.","family":"Kov\u00e1cs","sequence":"additional","affiliation":[{"name":"Sanford Children\u2019s Health Research Center, Sanford Research\/USD, Sioux Falls, SD, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David A.","family":"Pearce","sequence":"additional","affiliation":[{"name":"Sanford Children\u2019s Health Research Center, Sanford Research\/USD, Sioux Falls, SD, USA"},{"name":"Department of Pediatrics, Sanford School of Medicine of the University of South Dakota, Sioux Falls, SD, 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