Figure 1.

(A) Schematic representation of central excitatory and inhibitory synapses and main antibody targets. The proteins targeted by antibodies associated with autoimmune encephalitis are proteins expressed on the neuronal surface, often at both presynaptic and postsynaptic levels on inhibitory (GABAergic) and/or excitatory (glutamatergic) neurons in the central nervous system (CNS). (B) Schematic representation of CASPR2. CASPR2 localizes at the juxtaparanode of myelinated axons. CASPR2 binds to contactin-2/TAG-1 via its extracellular domain and links to PDZ-binding proteins and to the cytoskeleton via protein 4.1B, stabilizing Kv1 channels [adapted with permission from Giannoccaro et al. (2)].