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Table 1 Epitope target(s) of CNS and PNS human autoantibodies

From: Mapping autoantigen epitopes: molecular insights into autoantibody-associated disorders of the nervous system

  CNS/PNS disorder Antigen Target: extra/intra Main epitope Other epitopes Number of N-glycosylation sites Sample type Methodology Reference
CNS Anti-NMDAR encephalitis Neuronal NMDAR Extra GluN1/NR1 subunit (ATD, N368/G369) 10 (N368a) CSF Subcloning/ICC Gleichman et al. [35]
SLE Extra NR2A/2B subunit (LBD/S1, aa283–287)   6 Sera/CSF Phage display peptide library DeGiorgio et al. [67].
Anti-AMPAR encephalitis Neuronal AMPAR Extra GluR1 subunit (Bottom of ATD) S1 domain 6 Sera/CSF Subcloning/ICC, Fusion protein-based western blot Gleichman et al. [32]
Autoimmune encephalitis Neuronal Caspr2 Extra Disc domain Lam1, Lam2, Egf1 domains 12 Sera Subcloning/ICC, western blot Olsen et al. [37]
Extra Disc, Lam1 domains Sera/CSF Flow cytometry cell-based assay Pinatel et al. [38]
Encephalitis/SPS Neuronal GAD65 Intra Catalytic region (aa221-444) C-terminal domain 0 Sera Immunoprecipitation assay Fouka et al. [48]
Intra aa4-22, aa308-365, aa451-585 Sera Competition assay Raju et al. [47]
NMOSD/BON/ADEM/CRION Oligodendrocytes - MOG Extra β strand/CC’-loop (P42) FG-loop (H103/S104) 1 (N60a) Sera Subcloning/cell-bound reactivity assay Mayer et al. [56]
NMO Astrocytes - AQP4 isoform M23 Extra Loop A (aa61-64) Loop C (aa146-150)
Loop E (W227)
2 Sera Subcloning/ICC Pisani et al. [62]
Intra aa1-22, Loop B (aa88-113), aa252-275 Sera ELISA Kampylafka et al. [61]
Extra Loop A (aa66-69) Loop C (N153), Loop E (H230) Sera/CSF Cell-based assay Tuller et al. [65]
PNS MG Muscle - AChR Extra α1 subunit (aa67-76) 1 Sera Solid-phase radioimmunoassay Tzartos et al. [33]
Extra α1 subunit (aa1-14, aa67-76) Sera Radioimmunoassay Luo et al. [79]
Extra α1 subunit (N68, D71) Sera Peptide competition binding assay Papadouli et al. [80]
Wahlsten et al. [81]
Intra α1 subunit (aa373–380), β1 subunit (aa354–359) Sera Competition assay Tzartos et al. [82]
MG Muscle - MuSK Extra Ig-like domain 1 Ig-like domain 2, Ig-like domain 3/ Fz-like domain 2 Sera Radioimmunoprecipitation assay McConville et al. [84]
Extra Ig-like domain 1, Ig-like domain 2 Sera Radioimmunoprecipitation assay Ohta et al. [85]
Extra Ig-like domain 1 Ig-like domain 2 Sera Competition binding, ELISA, subcloning/ICC Huijbers et al. [83]
Extra Ig-like domain 1 Ig-like domain 2, Fz-like domain Sera ELISA Huijbers et al. [34]
MG Titin Intra Ig C2-like domain, Fn-like domain (MGT30) 0 Sera cDNA expression library Gautel et al. [88]
LEMS Neuronal
P/Q-type VGCC
Extra α1 subunit (linker region of S5-6 in repeated domains II, III, IV) β1 subunit 1 Sera Radioimmunoprecipitation assay Parsons et al. [94]
Extra α1 subunit (linker region of domain IV) α1 subunit (linker region of domain II) Sera Immunoprecipitation assay Takamori et al. [95]
Extra α1 subunit (linker region of domain III) Sera Immunoblot Iwasa et al. [96]
NMT Neuronal Caspr2 Extra Disc domain Lam1, Lam2, Egf1 12 Sera Subcloning/ICC, Western blot Olsen et al. [37]
Extra Disc, Lam1 domains Sera/CSF Flow cytometry cell-based assay Pinatel et al. [38]
NPSLE Neuronal NMDAR Extra GluN1/NR1 (aa19-44, aa56-81) 10 Sera/CSF ELISA Ogawa et al. [72]
CIDP, GBS, AMAN, AIPD Neuronal NF186 Extra Fn V domain, Mucin-like domain 8 Sera Cell-based assay Ng et al. [107]
Glial NF155 Extra Fn III domain, Fn IV domain
CIDP, GBS Neuronal Contactin Extra Ig C2-like domain Vb 9 Sera Subcloning/ICC Labasque et al. [108]
  1. AChR acetylcholine receptor, AIPD acute inflammatory demyelinating polyradiculoneuropathy, AMAN acute motor axonal neuropathy, AMPAR α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor, ADEM acute disseminated encephalomyelitis, AQP4 aquaporin-4, BON bilateral optic neuritis, Caspr2 contactin-associated protein-like 2, CIDP chronic inflammatory demyelinating polyneuropathy, CNS central nervous system, CRION chronic relapsing inflammatory optic neuropathy, CSF cerebrospinal fluid, DNA deoxyribonucleic acid, ELISA enzyme-linked immunosorbent assay, Fn fibronectin-type III, Fz Frizzled, GAD65 65 kDa glutamic acid decarboxylase, GBS Guillain-Barre syndrome, ICC immunocytochemistry, Ig immunoglobulin, LBD ligand binding domain, LEMS Lambert-Eaton myasthenic Syndrome, Lgi1 leucine-rich glioma-inactivated protein 1, MG myasthenia gravis, MOG myelin oligodendrocyte glycoprotein, MS multiple sclerosis, MuSK muscle-specific kinase, NF neurofascin, NMDAR N-methyl-D-aspartate receptor, NMO neuromyelitis optica, NMOSD neuromyelitis optica syndrome disorder, NMT acquired neuromyotonia, NPSLE neuropsychiatric systematic lupus erythematosus, PNS peripheral nervous system, SPS Stiff Person Syndrome, T1D type 1 Diabetes, VGCC voltage-gated calcium channel, VGKC voltage-gated potassium channel, - not applicable
  2. aPotentially important glycosylation site involved in antibody binding and formation of epitope
  3. bN-glycosylation sites N467/N473/N494 involved in antibody binding