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Data from: Glutamate receptor delta2 serum antibodies in paediatric opsoclonus myoclonus ataxia syndrome

Data from: Glutamate receptor delta2 serum antibodies in paediatric opsoclonus myoclonus ataxia syndrome
Data from: Glutamate receptor delta2 serum antibodies in paediatric opsoclonus myoclonus ataxia syndrome
Objective: To identify neuronal surface antibodies in opsoclonus myoclonus ataxia syndrome (OMAS) usingcontemporary antigen discovery methodology. Methods: OMAS patient serum IgG immunohistochemistry using age-equivalent rat cerebellar tissue was followed by immunoprecipitation, gel electrophoresis and mass spectrometry. Data are available via ProteomeXchange (identifier PXD009578). This generated a list of potential neuronal surface cerebellar autoantigens. Live cell-based assays (CBA) were used to confirm membrane-surface antigens and adsorb antigen-specific IgGs. The serological results were compared to the clinical data. Results: Four of the six OMAS sera tested bound rat cerebellar sections. Two of these sera with similar immunoreactivities were used in immunoprecipitation experiments using cerebellum from postnatal rat pups (P18). Mass spectrometry identified 12 cell-surface proteins, of which glutamate receptor delta 2 (GluD2), a predominately cerebellar-expressed protein, was found at a threefold higher concentration than the other 11 proteins. Antibodies to GluD2 were identified in 14/16 (87%) OMAS samples, compared with 5/139 (5%) pediatric and 1/38 (2.6%) adult serum controls (p<0.0001), and in 2/4 sera from patients with neuroblastoma without neurological features. Adsorption of positive OMAS sera against GluD2-transfected cells substantially reduced but did not eliminate, reactivity towards cerebellar sections. Conclusion: Autoantibodies to GluD2 are frequent in patients with OMAS, bind to surface determinants and are potentially pathogenic.
immunotherapy, paraneoplastic, opsoclonus myoclonus ataxia syndrome (OMAS), neuroblastoma, GluD2, Autoimmune, encephalitis, cerebellum
Dryad
Berridge, Georgina
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Menassa, David
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Moloney, Teresa
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Waters, Patrick J
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Welding, Imogen
52c89ba0-6363-4492-a5a4-0e844b280a92
Thomsen, Selina
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Zuberi, Sameer
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Fischer, Roman
74a35b05-e61e-4a70-ad51-f72f189f4d31
Aricescu, A. Radu
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Pike, Michael
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Dale, Russell C
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Kessler, Benedikt
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Vincent, Angela
eff75b70-2b05-411a-8323-5a9a2d4c64d2
Lim, Ming
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Irani, Sarosh R
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Lang, Bethan
ebdbe496-cc0c-4aeb-a5dc-380d6a786686
Berridge, Georgina
d4606ead-aadb-4e09-aabf-93b9827fda5b
Menassa, David
eeb394a6-c72b-49d7-a820-95b0256c22d5
Moloney, Teresa
e213675f-d2e8-4db3-9db6-4c4c11a0e285
Waters, Patrick J
45ff5ab3-8628-475e-82cd-15fbe70ea0de
Welding, Imogen
52c89ba0-6363-4492-a5a4-0e844b280a92
Thomsen, Selina
64dc85d6-a599-436b-881a-40f792c686f4
Zuberi, Sameer
f909ca9d-b2bb-4cc0-9d36-38059c70e8ee
Fischer, Roman
74a35b05-e61e-4a70-ad51-f72f189f4d31
Aricescu, A. Radu
f4189c11-458d-4b88-85b6-1b0fabbe881f
Pike, Michael
6ba76f78-f83f-4be5-a6c4-ef62b4e89646
Dale, Russell C
53f4945a-7486-4187-8e07-d3a3ddd5cd3f
Kessler, Benedikt
603ee4ec-d269-4520-918b-c2d34be8553a
Vincent, Angela
eff75b70-2b05-411a-8323-5a9a2d4c64d2
Lim, Ming
d81771dc-9c5e-4cdb-a87a-3cd4a410368a
Irani, Sarosh R
7fcadbba-aa80-4640-bf5f-2b5dfb6bfcd5
Lang, Bethan
ebdbe496-cc0c-4aeb-a5dc-380d6a786686

Berridge, Georgina, Menassa, David, Moloney, Teresa, Waters, Patrick J, Welding, Imogen, Thomsen, Selina, Zuberi, Sameer, Fischer, Roman, Aricescu, A. Radu, Pike, Michael, Dale, Russell C, Kessler, Benedikt, Vincent, Angela, Lim, Ming, Irani, Sarosh R and Lang, Bethan (2018) Data from: Glutamate receptor delta2 serum antibodies in paediatric opsoclonus myoclonus ataxia syndrome. Dryad doi:10.5061/DRYAD.TQ61224 [Dataset]

Record type: Dataset

Abstract

Objective: To identify neuronal surface antibodies in opsoclonus myoclonus ataxia syndrome (OMAS) usingcontemporary antigen discovery methodology. Methods: OMAS patient serum IgG immunohistochemistry using age-equivalent rat cerebellar tissue was followed by immunoprecipitation, gel electrophoresis and mass spectrometry. Data are available via ProteomeXchange (identifier PXD009578). This generated a list of potential neuronal surface cerebellar autoantigens. Live cell-based assays (CBA) were used to confirm membrane-surface antigens and adsorb antigen-specific IgGs. The serological results were compared to the clinical data. Results: Four of the six OMAS sera tested bound rat cerebellar sections. Two of these sera with similar immunoreactivities were used in immunoprecipitation experiments using cerebellum from postnatal rat pups (P18). Mass spectrometry identified 12 cell-surface proteins, of which glutamate receptor delta 2 (GluD2), a predominately cerebellar-expressed protein, was found at a threefold higher concentration than the other 11 proteins. Antibodies to GluD2 were identified in 14/16 (87%) OMAS samples, compared with 5/139 (5%) pediatric and 1/38 (2.6%) adult serum controls (p<0.0001), and in 2/4 sera from patients with neuroblastoma without neurological features. Adsorption of positive OMAS sera against GluD2-transfected cells substantially reduced but did not eliminate, reactivity towards cerebellar sections. Conclusion: Autoantibodies to GluD2 are frequent in patients with OMAS, bind to surface determinants and are potentially pathogenic.

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More information

Published date: 2018
Keywords: immunotherapy, paraneoplastic, opsoclonus myoclonus ataxia syndrome (OMAS), neuroblastoma, GluD2, Autoimmune, encephalitis, cerebellum

Identifiers

Local EPrints ID: 436511
URI: http://eprints.soton.ac.uk/id/eprint/436511
PURE UUID: 10bae9fc-b62e-48fc-9aa9-8a681834a207

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Date deposited: 11 Dec 2019 17:31
Last modified: 03 Jan 2024 16:07

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Contributors

Creator: Georgina Berridge
Creator: David Menassa
Creator: Teresa Moloney
Creator: Patrick J Waters
Creator: Imogen Welding
Creator: Selina Thomsen
Creator: Sameer Zuberi
Creator: Roman Fischer
Creator: A. Radu Aricescu
Creator: Michael Pike
Creator: Russell C Dale
Creator: Benedikt Kessler
Creator: Angela Vincent
Creator: Ming Lim
Creator: Sarosh R Irani
Creator: Bethan Lang

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