{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,29]],"date-time":"2026-10-29T14:44:54Z","timestamp":1793285094399,"version":"4.5.0"},"reference-count":44,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2020,9,28]],"date-time":"2020-09-28T00:00:00Z","timestamp":1601251200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Neuropathology Appl Neurobio"],"published-print":{"date-parts":[[2021,2]]},"abstract":"<jats:sec><jats:title>Aims<\/jats:title><jats:p>Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a life\u2010threatening condition often associated with highly raised serum antibodies to glycine receptors (GlyRs); these bind to the surface of large neurons and interneurons in rodent brain and spinal cord sections and, <jats:italic>in vitro<\/jats:italic>, inhibit function and reduce surface expression of the GlyRs. The effects <jats:italic>in vivo<\/jats:italic> have not been reported.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Purified plasma IgG from a GlyR antibody\u2010positive patient with PERM, and a healthy control (HC), was injected daily into the peritoneal cavity of mice for 12\u00a0days; lipopolysaccharide (LPS) to open the blood\u2013brain barrier, was injected on days 3 and 8. Based on preliminary data, behavioural tests were only performed 48\u00a0h post\u2010LPS on days 5\u20137 and 10\u201312.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The GlyR IgG injected mice showed impaired ability on the rotarod from days 5 to 10 but this normalized by day 12. There were no other behavioural differences but, at termination (d13), the GlyR IgG\u2010injected mice had IgG deposits on the neurons that express GlyRs in the brainstem and spinal cord. The IgG was not only on the surface but also inside these large GlyR expressing neurons, which continued to express surface GlyR.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Despite the partial clinical phenotype, not uncommon in passive transfer studies, the results suggest that the antibodies had accessed the GlyRs in relevant brain regions, led to antibody\u2010mediated internalization and increased GlyR synthesis, compatible with the temporary loss of function.<\/jats:p><\/jats:sec>","DOI":"10.1111\/nan.12666","type":"journal-article","created":{"date-parts":[[2020,9,21]],"date-time":"2020-09-21T07:25:25Z","timestamp":1600673125000},"page":"316-327","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Systemic delivery of human GlyR IgG antibody induces GlyR internalization into motor neurons of brainstem and spinal cord with motor dysfunction in 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