Effects ofIgLON5Antibodies on Neuronal Cytoskeleton: A Link between Autoimmunity and Neurodegeneration

被引:66
|
作者
Landa, Jon [1 ]
Gaig, Carles [1 ,2 ]
Planaguma, Jesus [1 ]
Saiz, Albert [1 ,2 ]
Antonell, Anna [3 ]
Sanchez-Valle, Raquel [3 ]
Dalmau, Josep [1 ,2 ,4 ,5 ]
Graus, Francesc [1 ]
Sabater, Lidia [1 ]
机构
[1] Univ Barcelona, Inst Invest Biomed August Pi & Sunyer IDIBAPS, Neuroimmunol Program, Barcelona, Spain
[2] Hosp Clin Barcelona, Serv Neurol, Barcelona, Spain
[3] Univ Barcelona, Alzheimers Dis & Other Cognit Disorders Unit, Neurol Dept, Hosp Clin,IDIBAPS, Barcelona, Spain
[4] Univ Penn, Dept Neurol, Philadelphia, PA 19104 USA
[5] Inst Catalana Recerca & Estudis Avancats ICREA, Barcelona, Spain
关键词
D O I
10.1002/ana.25857
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
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
引用
收藏
页码:1023 / 1027
页数:5
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