{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,23]],"date-time":"2026-07-23T03:25:36Z","timestamp":1784777136131,"version":"3.55.0"},"reference-count":90,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2020,9,2]],"date-time":"2020-09-02T00:00:00Z","timestamp":1599004800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J of Comparative Neurology"],"published-print":{"date-parts":[[2021,4,15]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Lafora disease (LD) is a genetic and fatal form of neurodegenerative disorder characterized by myoclonic epilepsy and cognitive deficits. LD is caused by loss\u2010of\u2010function mutations in the\n                    <jats:italic>EPM2A<\/jats:italic>\n                    or the\n                    <jats:italic>NHLRC1<\/jats:italic>\n                    gene. A major hallmark of LD is the presence of abnormal glycogen aggregates in neurons and other tissues. Functional studies on the genes have, therefore, mostly focused on glycogen metabolism. The physiological basis of cognitive deficits in LD is thus largely unexplored. Alterations in dendritic spine morphology are known in neurodevelopmental and neuropsychiatric disorders. We, therefore, analyzed the dendritic spine morphologies in pyramidal neurons of the hippocampal and Cortical layer V of the\n                    <jats:italic>Epm2a<\/jats:italic>\n                    or\n                    <jats:italic>Nhlrc1<\/jats:italic>\n                    knockout mice brain. We found a significant increase in the density, length, and reduction in the width of the dendritic spines in Postnatal day 21 to 12\u2010month\u2010old LD animals. Similar observations were made in the primary cultures of neurons derived from the hippocampi of the embryonic brain, suggesting that the aberrant spine phenotype could be a developmental defect in LD. We also looked at the cognitive and behavioral deficits as a possible readout of the spine abnormalities. The LD animals exhibited hyperactivity, reduced anxiety\u2010like behavior, and deficits in the spatial and nonspatial memory. Such abnormalities were seen in the younger (1\u20132 months) as well as the older (7\u20138 months) age groups. Taken together, our results suggest that the dendritic spine abnormalities are primary developmental defects in the LD model and these defects might underlie some of the symptoms, including cognitive deficits, in LD.\n                  <\/jats:p>","DOI":"10.1002\/cne.25006","type":"journal-article","created":{"date-parts":[[2020,8,12]],"date-time":"2020-08-12T09:30:47Z","timestamp":1597224647000},"page":"1099-1120","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Dendritic spine abnormalities correlate with behavioral and cognitive deficits in mouse models of Lafora disease"],"prefix":"10.1002","volume":"529","author":[{"given":"Komal","family":"Taneja","sequence":"first","affiliation":[{"name":"Department of Biological Sciences and Bioengineering Indian Institute of Technology Kanpur  Kanpur Uttar Pradesh 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