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Effects of IgLON5 Antibodies on Neuronal Cytoskeleton: A Link between Autoimmunity and Neurodegeneration
Author(s) -
Landa Jon,
Gaig Carles,
Plagumà Jesús,
Saiz Albert,
Antonell Ana,
SanchezValle Raquel,
Dalmau Josep,
Graus Francesc,
Sabater Lidia
Publication year - 2020
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.25857
Subject(s) - neurodegeneration , cytoskeleton , neurofilament , biology , neurite , neuroscience , autoimmunity , microbiology and biotechnology , antibody , pathology , medicine , immunology , disease , cell , genetics , in vitro , immunohistochemistry
Anti‐IgLON5 disease is a neurological disorder characterized by autoantibodies against IgLON5 and pathological evidence of neuronal‐specific tau accumulation. Here, we report that patients' IgLON5 IgG, but not other cell‐surface antibodies, disrupt the cytoskeletal organization in cultured rat hippocampal neurons, resulting in dystrophic neurites and axonal swelling. Adsorption of IgLON5 IgG with HEK293 cells expressing IgLON5 abrogated the indicated cytoskeletal changes. These findings, along with an increase of levels of neurofilaments in patients' cerebrospinal fluid, suggest that IgLON5 IgG, unlike other cell‐surface antibodies, disrupts neuronal cytoskeleton maintenance, providing a link between autoimmunity and neurodegeneration. ANN NEUROL 2020;88:1023–1027