1. Nat. Rev. Neurol. doi:10.1038/nrneurol.2017.84
Figure 3 Immunological findings in patients with CIDP and anti-NF155 antibodies
Figure 3 | Immunological findings in patients with CIDP and anti-NF155 antibodies. a | Serum from a patient with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) contains autoantibodies that target neurofascin splice variant 155 (NF155, red fluorescence), which co-localize at the paranode with a commercially available antibody targeting contactin-1 (CNTN1, green fluorescence). The merging of both fluorescent signals appears as yellow staining at the paranodes in teased nerve fibres, suggesting perfect co-localization of red (serum antibody) and green (commercial antibody). a–c | Cell nuclei are indicated by staining with 4',6-diamidino-2-phenylindole (DAPI; blue fluorescence). b | NF155 is expressed by myelinating oligodendrocytes in vitro. Serum from a patient with CIDP contains anti-NF155 autoantibodies (red fluorescence), which bind to oligodendrocytes expressing myelination markers such as myelin basic protein (MBP, green fluorescence). c | Transfection of an expression vector encoding human NF155 protein has become the gold standard for anti-NF155 detection. Main image: anti-NF155 antibodies (red fluorescence) from a patient with CIDP bind to the surface of NF155-transfected human embryonic kidney (HEK)293 cells. Inset image: patient-derived anti-NF155 autoantibodies (red fluorescence) co-localize with a commercially available anti-NF155 antibody (green fluorescence). Merger of both stains appears as a yellow signal. d | Enzyme-linked immunosorbent assay (ELISA) using human recombinant NF155 protein is a highly specific test to confirm anti-NF155 positivity in patients with CIDP. Only sera from anti-NF155+ patients will react with the recombinant NF155 protein; thus, optical density is close to zero in anti-NF155− patients with CIDP as well as in healthy controls (of note, using other versions of NF155 protein (rat-derived, or fusion proteins) in such tests can lead to nonspecific binding and false positives). e | Finally, in patients with anti-NF155 antibodies, IgG isotype determination is achieved by ELISA. All patients tested had minor amounts of other IgG isotypes but IgG4 is clearly the predominant anti-NF155 isotype in these patients. All scale bars 10 µm; optical density, a measure of the intensity of the ELISA colorimetric reaction.
Querol, L. et al. (2017) Autoantibodies in chronic inflammatory neuropathies: diagnostic and therapeutic implications
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