Fig. 1

Intra-myonuclei stained by anti-p62 antibody. H&E (a, c, e, g, i) and p-62 antibody (b, d, f, h, j) staining of serial sections of the muscle sample for each disease. Excluded staining pattern of anti-p62 antibody in the control (k), GNE myopathy (l), and IBM (m) samples. n The graph shows the frequency of p62-positive intra-myonuclei in each disease. Error bars indicate mean ± SEM. ***p < 0.0001; ns no significance. o PABPN1-positive aggregates were localized in the myonuclei, similar to p62 positive myo-INIs in OPMD. a–m, o) All bars show 10 µm. ***OPMD shows higher frequency of p62-positive intra-myonuclei compared to OPDM_LRP12, OPDM_GIPC1, OPDM_NOTCH2NLC, NIID, IBM, GNE, IBMPFD, DES-related myopathy, and control samples (p < 0.0001). H&E hematoxylin and eosin; IBM: inclusion body myositis; OPMD oculopharyngeal muscular dystrophy; OPDM oculopharyngodistal myopathy; NIID neuronal intranuclear inclusion disease; IBMPFD inclusion body myopathy with Paget’s disease of bone and frontotemporal dementia