Autoantibodies Against Axonal Neurofilaments in Patients with Kuru and Creutzfeldt-Jakob Disease

• DOI: 10.1126/science.6997994
• Publication Date: 2006-10-05T17:45:58Z
• Authors: J. Sotelo; C. J. Gibbs; D. C. Gajdusek
• Keywords: 0301 basic medicine; 03 medical and health sciences; Kuru; Antibody Specificity; Fluorescent Antibody Technique; Humans; Axons; Creutzfeldt-Jakob Syndrome; Cytoskeleton; Autoantibodies; Autoimmune Diseases; 3. Good health

The serums of some patients with subacute spongiform encephalopathies contain an autoantibody in high titer against a normal fibrillar protein within the axon of mature central neurons in culture. The morphological features of this neurofilament, as demonstrated by immunofluorescence and immunoperoxidase staining, and the partial characterization of the antibody are described. The detection of this hetero-specific autoantibody is the first evidence of an immune reaction in the spongiform encephalopathies.