Protein expression was analyzed by serial staining of sections from biopsies of all GNE myopathy patients with immunohistochemical double labeling of 1) APP and MHC-I, 2) αB-crystallin and NCAM, 3) IL-1β and β-amyloid, and 4) iNOS and P-neurofilament. This evidence concerns the gene NOS2 and Distal myopathy, Nonaka type.