Fig. 2

Intra-nuclear inclusions of non-muscle cells in muscle biopsy. H&E (a, c, e, g, i, k, m, o, q, s, u, w) and p62 (b, d, f, h, j, l, n, p, r, t, v, x) staining of serial sections of the muscle sample for each disease. The p62-positive INIs are observed in Schwann cells, pericytes, and/or perineurium cells (b, h, n, t), blood vessels (d, j, p, v), and muscle spindles (f, l, r). The p62-positive INIs are not seen in the muscle spindles from IBM (w, x). a–x All black bars show 20 µm. y Frequency of p62-positive INIs in non-muscle cells in total for each disease. Error bars indicate mean ± SEM. *, p < 0.05; ns, no significance. z The muscle spindle taken from OPDM_NOTCH2NLC shows p62-positive INIs (red color). H&E hematoxylin and eosin; INIs intra-nuclear inclusions; IBM inclusion body myositis; OPDM oculopharyngodistal myopathy; NIID neuronal intranuclear inclusion disease; IBMPFD inclusion body myopathy with Paget’s disease of bone and frontotemporal dementia; OPMD oculopharyngeal muscular dystrophy