{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,20]],"date-time":"2026-09-20T01:39:57Z","timestamp":1789868397378,"version":"4.0.1"},"reference-count":39,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2024,2,2]],"date-time":"2024-02-02T00:00:00Z","timestamp":1706832000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100004587","name":"Instituto de Salud Carlos III","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100004587","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Anti-IgLON5 disease is a neurological disorder characterized by autoantibodies against IgLON5 and pathological evidence of neurodegeneration. IgLON5 is a cell adhesion molecule of unknown function that is highly expressed in the brain. Our aim was to investigate the impact of IgLON5 loss-of-function in evaluating brain morphology, social behavior, and the development of symptoms observed in an IgLON5 knockout (IgLON5-KO) mouse model.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>The IgLON5-KO mice were generated using CRISPR-Cas9 technology. Immunohistochemistry on fixed sagittal brain sections and Western blotting brain lysates were used to confirm IgLON5 silencing and to evaluate the presence of other cell surface proteins. Two- month-old IgLON5-KO and wild-type (WT) mice underwent a comprehensive battery of behavioral tests to assess 1) locomotion, 2) memory, 3) anxiety, 4) social interaction, and 5) depressive-like behavior. Brain sections were examined for the presence of anatomical abnormalities and deposits of hyperphosphorylated tau in young adult (2-month-old) and aged (22-month-old) mice.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Mice did not develop neurological symptoms reminiscent of those seen in patients with anti-IgLON5 disease. Behavioral testing revealed that 2-month-old IgLON5-KO mice showed subtle alterations in motor coordination and balance. IgLON5-KO females exhibited hyperactivity during night and day. Males were observed to have depressive-like behavior and excessive nest-building behavior. Neuropathological studies did not reveal brain morphological alterations or hyperphosphorylated tau deposits.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>IgLON5-KO mice showed subtle alterations in behavior and deficits in fine motor coordination but did not develop the clinical phenotype of anti-IgLON5 disease.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fimmu.2024.1347948","type":"journal-article","created":{"date-parts":[[2024,2,1]],"date-time":"2024-02-01T23:54:28Z","timestamp":1706831668000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":6,"title":["IgLON5 deficiency produces behavioral alterations in a knockout mouse model"],"prefix":"10.3389","volume":"15","author":[{"given":"Jon","family":"Landa","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana Beatriz","family":"Serafim","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mercedes","family":"Alba","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Estibaliz","family":"Maudes","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Molina-Porcel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Garcia-Serra","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesco","family":"Mannara","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Josep","family":"Dalmau","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesc","family":"Graus","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lidia","family":"Sabater","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2024,2,2]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(14)70051-1","article-title":"A novel non-rapid-eye movement and rapid-eye-movement parasomnia with sleep breathing disorder associated with antibodies to IgLON5: A case series, characterisation of the antigen, and post-mortem study","volume":"13","author":"Sabater","year":"2014","journal-title":"Lancet Neurol"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000003887","article-title":"Clinical manifestations of the anti-IgLON5 disease","volume":"88","author":"Gaig","year":"2017","journal-title":"Neurology"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000012639","article-title":"Frequency and characterization of movement disorders in anti-igLON5 disease","volume":"97","author":"Gaig","year":"2021","journal-title":"Neurology"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1111\/cns.13038","article-title":"Motor neuron disease-like phenotype associated with anti-IgLON5 disease","volume":"24","author":"Tao","year":"2018","journal-title":"CNS Neurosci Ther"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-022-11262-0","article-title":"Motor-neuron-disease-like phenotype associated with IgLON5 disease","volume":"269","author":"Sista","year":"2022","journal-title":"J Neurol"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra1708712","article-title":"Antibody-mediated encephalitis","volume":"378","author":"Dalmau","year":"2018","journal-title":"New Engl J Med vol"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-016-1591-8","article-title":"Neuropathological criteria of anti-IgLON5-related tauopathy","volume":"132","author":"Gelpi","year":"2016","journal-title":"Acta Neuropathol."},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1212\/NXI.0000000000000605","article-title":"HLA and microtubule-associated protein tau H1 haplotype associations in anti-IgLON5 disease","volume":"6","author":"Gaig","year":"2019","journal-title":"Neurol - Neuroimmunol. 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