Node of Ranvier disruption: A key pathophysiology in immune-mediated neuropathies

Abstract

One important role of Schwann cells is to promote clustering of voltage-gated Na+ channels on the axolemma at the nodes of Ranvier. Recent cumulative evidence demonstrates that disruption of nodal Na+ channel clusters is the key pathophysiology in immune-mediated neuropathies such as Guillain-Barré syndrome. Autoimmune attack against either axons or myelin can disturb neuron-Schwann cell interactions at and near nodes, disrupting Na+ channel clusters and resulting in nerve conduction block. Furthermore, the proteins highly accumulated at and near nodes can be a direct target of autoimmune reactions. This chapter focuses on dysfunction and disruption of nodes of Ranvier during autoimmune neuropathies.