Neurofascin 155 IgG4 Autoantibody
Neurofascin-155 (NF155) is a paranodal protein of importance for the function of the node of Ranvier. Neurofascin 155 is expressed in both the central and peripheral nervous systems.
The primary effect of IgG4 anti-NF155 antibodies is likely to be the blockade of interactions between NF155 and CNTN1/CASPR1, which leads to Schwann cell terminal loop detachment from axons at paranodes.
IgG4 autoantibodies against NF155 have been identified in 4-18% of patients with Chronic Inflammatory Demyelinating Polyneuropathy (CIDP). This subgroup of patients is characterized by younger age at onset (20-30 years), ataxia, gait disturbance, tremor, and distal weakness. They are often refractory to treatment with intravenous immunoglobulin (IVIg) or steroids but may respond to plasmapheresis or rituximab
Reference
Anti-Neurofascin 155 Antibody-Positive Chronic Inflammatory Demyelinating Polyneuropathy/Combined Central and Peripheral Demyelination: Strategies for Diagnosis and Treatment Based on the Disease Mechanism, Front Neurol June 10, 2021; https://doi.org/10.3389/fneur.2021.665136.
