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000162857 0247_ $$2ISSN$$a1540-9358
000162857 0247_ $$2ISSN$$a1540-9538
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000162857 0247_ $$2doi$$a10.1084/jem.20210012
000162857 037__ $$aDZNE-2021-01512
000162857 041__ $$aEnglish
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000162857 1001_ $$0P:(DE-2719)2811468$$aKreye, Jakob$$b0$$eFirst author
000162857 245__ $$aEncephalitis patient-derived monoclonal GABAA receptor antibodies cause epileptic seizures.
000162857 260__ $$aNew York, NY$$bRockefeller Univ. Press$$c2021
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000162857 520__ $$aAutoantibodies targeting the GABAA receptor (GABAAR) hallmark an autoimmune encephalitis presenting with frequent seizures and psychomotor abnormalities. Their pathogenic role is still not well-defined, given the common overlap with further autoantibodies and the lack of patient-derived mAbs. Five GABAAR mAbs from cerebrospinal fluid cells bound to various epitopes involving the α1 and γ2 receptor subunits, with variable binding strength and partial competition. mAbs selectively reduced GABAergic currents in neuronal cultures without causing receptor internalization. Cerebroventricular infusion of GABAAR mAbs and Fab fragments into rodents induced a severe phenotype with seizures and increased mortality, reminiscent of encephalitis patients' symptoms. Our results demonstrate direct pathogenicity of autoantibodies on GABAARs independent of Fc-mediated effector functions and provide an animal model for GABAAR encephalitis. They further provide the scientific rationale for clinical treatments using antibody depletion and can serve as tools for the development of antibody-selective immunotherapies.
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000162857 650_2 $$2MeSH$$aAnimals
000162857 650_2 $$2MeSH$$aAntibodies, Monoclonal: immunology
000162857 650_2 $$2MeSH$$aAutoantibodies: immunology
000162857 650_2 $$2MeSH$$aAutoantigens: immunology
000162857 650_2 $$2MeSH$$aCells, Cultured
000162857 650_2 $$2MeSH$$aEncephalitis: immunology
000162857 650_2 $$2MeSH$$aEpilepsy: immunology
000162857 650_2 $$2MeSH$$aHEK293 Cells
000162857 650_2 $$2MeSH$$aHippocampus: immunology
000162857 650_2 $$2MeSH$$aHumans
000162857 650_2 $$2MeSH$$aMice
000162857 650_2 $$2MeSH$$aNeurons: immunology
000162857 650_2 $$2MeSH$$aReceptors, GABA-A: immunology
000162857 650_2 $$2MeSH$$aSeizures: immunology
000162857 7001_ $$00000-0002-5464-3779$$aWright, Sukhvir K$$b1
000162857 7001_ $$0P:(DE-2719)2814218$$aCasteren, Adriana$$b2
000162857 7001_ $$00000-0003-4724-5112$$aStöffler, Laura$$b3
000162857 7001_ $$0P:(DE-2719)2812697$$aMachule, Marie-Luise$$b4
000162857 7001_ $$0P:(DE-2719)2812526$$aReincke, Momsen$$b5
000162857 7001_ $$00000-0002-3678-5159$$aNikolaus, Marc$$b6
000162857 7001_ $$0P:(DE-2719)2812820$$aHoof, Scott$$b7
000162857 7001_ $$0P:(DE-2719)2812653$$aSanchez Sendin, Elisa$$b8
000162857 7001_ $$0P:(DE-2719)9001962$$aHomeyer, Marie Alice$$b9$$udzne
000162857 7001_ $$0P:(DE-2719)9001947$$aCordero Gomez, Cesar$$b10$$udzne
000162857 7001_ $$0P:(DE-2719)2811900$$aKornau, Hans-Christian$$b11
000162857 7001_ $$0P:(DE-2719)2810725$$aSchmitz, Dietmar$$b12
000162857 7001_ $$00000-0001-9454-206X$$aKaindl, Angela M$$b13
000162857 7001_ $$00000-0001-8777-4823$$aBoehm-Sturm, Philipp$$b14
000162857 7001_ $$00000-0002-5053-2211$$aMueller, Susanne$$b15
000162857 7001_ $$00000-0003-3317-9433$$aWilson, Max A$$b16
000162857 7001_ $$00000-0002-2032-1472$$aUpadhya, Manoj A$$b17
000162857 7001_ $$00000-0002-0363-627X$$aDhangar, Divya R$$b18
000162857 7001_ $$00000-0002-5038-5258$$aGreenhill, Stuart$$b19
000162857 7001_ $$00000-0003-1281-9008$$aWoodhall, Gavin$$b20
000162857 7001_ $$00000-0002-1305-6711$$aTurko, Paul$$b21
000162857 7001_ $$00000-0003-3214-2233$$aVida, Imre$$b22
000162857 7001_ $$0P:(DE-2719)2810922$$aGarner, Craig Curtis$$b23
000162857 7001_ $$00000-0002-5854-3759$$aWickel, Jonathan$$b24
000162857 7001_ $$00000-0002-9859-581X$$aGeis, Christian$$b25
000162857 7001_ $$00000-0001-7724-8643$$aFukata, Yuko$$b26
000162857 7001_ $$00000-0001-5200-9806$$aFukata, Masaki$$b27
000162857 7001_ $$0P:(DE-2719)2810931$$aPrüß, Harald$$b28$$eLast author
000162857 773__ $$0PERI:(DE-600)1477240-1$$a10.1084/jem.20210012$$gVol. 218, no. 11$$n11$$pe20210012$$tJournal of experimental medicine$$v218$$x0022-1007$$y2021
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