Anti–NF155 Antibodies
Test details
This laboratory assay identifies IgG (predominantly IgG4) autoantibodies against NF155. NF155 is expressed by Schwann cells at the paranodal region of peripheral nerves and is crucial for stabilising paranodal axo-glial junctions between the axon and myelin. These junctions separate the node of Ranvier from the internode and ensure efficient saltatory conduction. Anti-NF155 antibodies disrupt this interaction and normal nerve function, causing demyelination and impaired peripheral nerve conduction. Immunogenetically, anti-NF155 antibodies belong to subclass IgG4 are strongly associated with allele HLA-DRB1*15.
The disorder associated with anti-NF155 is a specific form of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), typically with earlier onset (often <40 years) and a phenotype of distal weakness, coarse low-frequency, high-amplitude tremor, and sensory ataxia; cerebellar signs and facial palsies may occur. Electrophysiology shows a demyelinating pattern with prolonged distal latencies and F-waves. CSF protein is frequently elevated, signalling inflammation. Response to IVIG is often limited, whereas rituximab and plasmapheresis tend to be more effective, with reduction in antibody titres and clinical improvement.
In general, the form of autoimmune neuropathy associated with anti-NF155 antibodies, together with those associated with anti-NF186, anti-CNTN1 and anti-CASPR1 antibodies, respectively, falls into the category of “nodopathies” or “autoimmune paranodopathies,” a group of autoimmune neuropathies characterized by autoantibodies against node and paranode adhesion molecules. They have clinical characteristics that are quite distinct from classic CIDP. None show clear signs of inflammation or macrophage-mediated demyelination, and all respond poorly to specific treatments for CIDP. This test is useful and essential in the differential diagnosis and therapeutic management of demyelinating neuropathies.
Sample type
Serum, plasma
Method
IFA cells
Preparation
Fasting for at least 8-12 hours before sampling
Storage conditions
Refer to the Health Service Charter to check storage conditions
Shipping
+2/+8°C
References
Johnson CB, Fehmi J, Rinaldi S. The immunology and neuropathology of the autoimmune nodopathies. J Neuroimmunol. 2025 Sep 15;406:578665. doi: 10.1016/j.jneuroim.2025.578665. Epub 2025 Jun 9. PMID: 40505341.
Zhang J, Hou X, Wei L, Liu J, Li S, Guo Y, Liu H, Jiang Y. Clinical characteristics of patients with autoimmune nodopathy with anti-neurofascin155 antibodies. Front Immunol. 2024 Apr 25;15:1345953. doi: 10.3389/fimmu.2024.1345953. PMID: 38726012; PMCID: PMC11079118.
Gupta P, Mirman I, Shahar S, Dubey D. Growing Spectrum of Autoimmune Nodopathies. Curr Neurol Neurosci Rep. 2023 May;23(5):201-212. doi: 10.1007/s11910-023-01264-4. Epub 2023 Apr 4. PMID: 37014546..
Martín-Aguilar L, Lleixà C, Pascual-Goñi E, Caballero-Ávila M, Martínez-Martínez L, Díaz-Manera J, Rojas-García R, Cortés-Vicente E, Turon-Sans J, de Luna N, Suárez-Calvet X, Gallardo E, Rajabally Y, Scotton S, Jacobs BC, Baars A, Cortese A, Vegezzi E, Höftberger R, Zimprich F, Roesler C, Nobile-Orazio E, Liberatore G, Hiew FL, Martínez-Piñeiro A, Carvajal A, Piñar-Morales R, Usón-Martín M, Albertí O, López-Pérez MÁ, Márquez F, Pardo-Fernández J, Muñoz-Delgado L, Cabrera-Serrano M, Ortiz N, Bartolomé M, Duman Ö, Bril V, Segura-Chávez D, Pitarokoili K, Steen C, Illa I, Querol L. Clinical and Laboratory Features in Anti-NF155 Autoimmune Nodopathy. Neurol Neuroimmunol Neuroinflamm. 2021 Nov 2;9(1):e1098. doi: 10.1212/NXI.0000000000001098. Erratum in: Neurol Neuroimmunol Neuroinflamm. 2021 Dec 20;9(1):e1129. doi: 10.1212/NXI.0000000000001129. PMID: 34728497; PMCID: PMC8564865.
M Van den Bergh PYK, van Doorn PA, Hadden RDM, Avau B, Vankrunkelsven P, Allen JA, Attarian S, Blomkwist-Markens PH, Cornblath DR, Eftimov F, Goedee HS, Harbo T, Kuwabara S, Lewis RA, Lunn MP, Nobile-Orazio E, Querol L, Rajabally YA, Sommer C, Topaloglu HA. European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint Task Force-Second revision. Eur J Neurol. 2021 Nov;28(11):3556-3583. doi: 10.1111/ene.14959. Epub 2021 Jul 30. Erratum in: Eur J Neurol. 2022 Apr;29(4):1288. doi: 10.1111/ene.15225. PMID: 34327760
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