Table 1.
Subject characteristics.
Table 2.
List of primary antibodies.
Figure 1.
CHMP2B-positive structures corresponding to RVs.
Modified Gomori trichrome staining, and immunohistochemical staining of serial sections using anti-charged multivesicular body protein 2B (CHMP2B) antibody, in an sporadic inclusion body myositis (s-IBM) case (case 1). Rimmed vacuoles (RVs) identified by modified Gomori trichrome staining (A) and the same structure immunopositive for CHMP2B (B). Scale bars = 20 µm.
Figure 2.
Immunohistochemistry for CHMP2B in muscle fibers of s-IBM and DMRV cases.
RVs in all s-IBM (A–C) and distal myopathy with RVs (DMRV) (D) cases detected by anti- CHMP2B antibody. Arrows indicate RVs. Scale bar = 20 µm.
Figure 3.
Immunohistochemistry for GVD markers in the Alzheimer's disease hippocampus.
Anti-CHMP2B (A), anti-caspase3 (B), anti-cyclin-dependent kinase 5 (CDK5) (C), anti-casein kinase 1δ (CK1δ) (D), anti-c-jun N-terminal kinase (JNK) (E), anti-leucine-rich repeat kinase 2 (LRRK2) (F), anti-annexin2 (G), anti-flotillin-1 (H), and anti-phosphorylated transactive response DNA binding protein-43 (pTDP43) (I). Arrows indicate granulovacuolar degeneration (GVD) bodies. Scale bars = 20 µm.
Figure 4.
Immunohistochemistry for GVD markers in muscle fibers of s-IBM cases.
Anti-CHMP2B (A), anti-caspase3 (B, C), anti-CDK5 (D), anti- CK1δ (E, F), anti-JNK (G, H), anti-LRRK2 (I), anti-annexin2 (J, K), anti-flotillin-1 (L, M), and anti-pTDP43 (N, O). (B, C), (E, F), (G,H), (J, K), (L, M) and (N, O) indicate 2 independent antibodies for the identification of each protein. Arrows indicate RVs. Scale bar = 20 µm.
Figure 5.
Representative confocal laser scanning micrograph.
Double immunofluorescence labeling and merged images in muscle sections from patients with s-IBM for CHMP2B (green) and pTDP43 (red) (A), caspase3 (green) and pTDP43 (red) (B), CDK5 (green) and pTDP43 (red) (C), CK1δ (green) and pTDP43 (red) (D), JNK (green) and pTDP43 (red) (E), LRRK2 (green) and pTDP43 (red) (F), annexin 2 (green) and pTDP43 (red) (G), and flotillin-1 (green) and pTDP43 (red) (H). Arrows indicate RVs. Scale bar = 20 µm.