Anti‐neurofascin antibody in combined central and peripheral demyelination. (28th November 2013)

• Record Type: Journal Article
• Authors: Yamasaki, Ryo
• Is Part Of: Clinical & experimental neuroimmunology. Volume 4(2013)Supplement 1
• Page Start: 68
• Page End: 75
• Publication Date: 2013-11-28
• Subjects: autoantibody -- chronic inflammatory demyelinating polyradiculoneuropathy -- combined central and peripheral demyelination -- immunoglobulin superfamily -- multiple sclerosis -- neurofascin; 616.80479
• DOI: 10.1111/cen3.12061
• Languages: English
• ISSNs: 1759-1961

Abstract

Abstract: Neurofascin (NF), a cell adhesion molecule expressed in both the central nervous system (CNS) and the peripheral nervous system (PNS), plays important roles in developing and maintaining neural structures. There are several subtypes of NF resulting from post‐translational modifications: NF155, 166, 180 and 186. Among them, NF155 and NF186 are expressed in the mature CNS/PNS. NF155 is present on the oligodendroglial cell surface in the CNS and on the Schwann cell surface in the PNS at paranodes, where it tightly connects with contactin and caspr on the axonal surface of the paranode, and acts as a stabilizer of the nodes of Ranvier. NF186 exists on the axonal surface at the nodes of Ranvier. NF186 is associated with voltage‐gated Na channels (Nav), whereas both NF186 and Nav are anchored by ankyrin G. NF186 contributes to the clustering of Nav at the node. Combined central and peripheral demyelination (CCPD) is an inflammatory demyelinating disorder affecting both the CNS and PNS tissues. Distinct mechanisms including multiple sclerosis and chronic inflammatory demyelinating polyradiculoneuropathy have been hypothesized to play a role in this condition on the basis of distinctive clinical and laboratory findings. We detected anti‐NF155 antibody by cell‐based assay, enzyme‐linked immunosorbent assay, and western blot in both the sera and cerebrospinal fluids of CCPD patients at high frequencies, but did not detect it in patients with other neurological disease or healthy controls. Herein, basic aspects and the clinical significance of NF as indispensable regulators and autoimmune target molecules are summarized.