Figure 4.
Number of rimmed vacuoles (RVs) and amyloid deposits after 6’-sialyllactose treatment. (A) Modified Gomori trichrome staining of gastrocnemius transverse sections. Arrowheads denote rimmed vacuoles. Immunosignals of amyloid-β1-40 and LC3 are localized within rimmed vacuoles. Scale bar = 50 μm. (B) Number of rimmed vacuoles. (C) Amyloid inclusions. (D and E) Measurement of amyloid-β1-40 (D) and amyloid-β1-42 (E) in muscle by ELISA. Control littermates (LM, closed circles, n = 5); non-treated GNE myopathy mice, (NT, open circles, n = 15); GNE myopathy mice with high dose 6’-sialyllactose (HD, upward closed triangles, n = 5); GNE myopathy mice with low dose 6’-sialyllactose (LD, downward closed triangles, n = 7); GNE myopathy mice with NeuAc (closed diamonds, n = 9).