{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,23]],"date-time":"2026-07-23T20:08:19Z","timestamp":1784837299691,"version":"3.55.0"},"reference-count":48,"publisher":"MDPI AG","issue":"14","license":[{"start":{"date-parts":[[2024,7,21]],"date-time":"2024-07-21T00:00:00Z","timestamp":1721520000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Anti-IgLON5 (IgLON5-IgG)-associated disease is a newly defined clinical entity. This literature review aims to evaluate its pathogenesis, which remains a pivotal question. Features that favour a primary neurodegenerative mechanism include the non-inflammatory tauopathy neuropathological signature and overrepresentation of microtubule-associated protein tau (MAPT) H1\/H1 genotype as seen in other sporadic tauopathies. In contrast, the cell-surface localisation of IgLON5, capability of anti-IgLON5 antibodies to exert direct in vitro pathogenicity and disrupt IgLON5 interactions with its binding partners, human leukocyte antigen (HLA)-DRB1*10:01 and HLA-DQB1*05:01 allele preponderance with high affinity binding of IgLON5 peptides, and responsiveness to immunotherapy favour a primary autoimmune process. The presentation and course of anti-IgLON5-associated disease is heterogenous; hence, we hypothesise that a multitude of immune mechanisms are likely simultaneously operational in this disease cohort.<\/jats:p>","DOI":"10.3390\/ijms25147956","type":"journal-article","created":{"date-parts":[[2024,7,23]],"date-time":"2024-07-23T07:57:23Z","timestamp":1721721443000},"page":"7956","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["IgLON5-IgG: Innocent Bystander or Perpetrator?"],"prefix":"10.3390","volume":"25","author":[{"ORCID":"https:\/\/orcid.org\/0009-0001-5322-3924","authenticated-orcid":false,"given":"Jane","family":"Andersen","sequence":"first","affiliation":[{"name":"Department of Immunology, NSW Health Pathology-ICPMR, Westmead Hospital, Sydney, NSW 2145, Australia"},{"name":"Faculty of Medicine and Health, The University of Sydney, Sydney, NSW 2050, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bronte","family":"Jeffrey","sequence":"additional","affiliation":[{"name":"Department of Immunology, NSW Health Pathology-ICPMR, Westmead Hospital, Sydney, NSW 2145, Australia"},{"name":"Faculty of Medicine, Western Sydney University, Sydney, NSW 2751, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Winny","family":"Varikatt","sequence":"additional","affiliation":[{"name":"Faculty of Medicine and Health, The University of Sydney, Sydney, NSW 2050, Australia"},{"name":"Department of Tissue Pathology and Diagnostic Oncology, NSW Health Pathology-ICPMR, Westmead Hospital, Sydney, NSW 2145, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Rodriguez","sequence":"additional","affiliation":[{"name":"Faculty of Medicine and Health, The University of Sydney, Sydney, NSW 2050, Australia"},{"name":"Douglass Hanly Moir Pathology, Sydney, NSW 2000, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ming-Wei","family":"Lin","sequence":"additional","affiliation":[{"name":"Department of Immunology, NSW Health Pathology-ICPMR, Westmead Hospital, Sydney, NSW 2145, Australia"},{"name":"Faculty of Medicine and Health, The University of Sydney, Sydney, NSW 2050, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1382-9419","authenticated-orcid":false,"given":"David A.","family":"Brown","sequence":"additional","affiliation":[{"name":"Department of Immunology, NSW Health Pathology-ICPMR, Westmead Hospital, Sydney, NSW 2145, Australia"},{"name":"Faculty of Medicine and Health, The University of Sydney, Sydney, NSW 2050, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,7,21]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"575","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":"ref_2","doi-asserted-by":"crossref","first-page":"e385","DOI":"10.1212\/NXI.0000000000000385","article-title":"IgLON5 antibody: Neurological accompaniments and outcomes in 20 patients","volume":"4","author":"Honorat","year":"2017","journal-title":"Neurol. 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