Neurofascin 155 (NF155) (NF155)

Neurofascin 155 (NF155) is a glial-specific cell adhesion molecule belonging to the immunoglobulin superfamily, localized specifically to the paranodal loops of myelinating glia (UniProt O94856). It is a critical component of the paranodal junction, where it associates with lipid rafts to facilitate stable interactions with the axonal Contactin-1/Caspr complex (PubMed: 11514521). This interaction is vital for anchoring the myelin sheath to the axon and maintaining the organization of the node of Ranvier, which is essential for rapid saltatory conduction in the nervous system. NF155 is recognized as a key autoantigen in a specific subset of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), often termed autoimmune nodopathy (PubMed: 24107218). Pathogenic IgG4 autoantibodies against NF155 disrupt the paranodal structure, leading to nerve conduction failure and clinical symptoms such as ataxia and tremor. Unlike typical CIDP, NF155-positive cases are often refractory to intravenous immunoglobulin (IVIG) but show significant clinical improvement when treated with B-cell depleting agents like Rituximab (PubMed: 25533448).

Other names

Neurofascin-155NFASCGlial neurofascinNF155 isoform

Therapeutic strategies focus on the depletion of B-cells or the removal of pathogenic IgG4 autoantibodies to prevent the disruption of the NF155-Contactin-1/Caspr complex at the paranodal junction.